Hematological disorder

Presented to
Dr. Amira Mohamed
by
Alaa Mohamed
Outlines
 Introduction
 Physical assessment for patient with chronic hematological disorder
 Common chronic hematological disorders
A. Red blood cell disorders
B. White blood cell disorders
 Leukopenia, lymphopenia, NEUTROPENIA
 Leukocytosis leukemia, lymphoma
C. Bleeding Disorders
 Identify hematopoiesis and the processes involved in maintaining
hemostasis.
 Thrombocytopenia
 Hemophilia
 Von Willebrand’s disease
 Disseminated intravascular coagulation (DIC)
coagulation disorder
 Thrombotic Disorders
 Therapies for Blood Disorders.
Introduction
 Hematology is defined as science that deal with the formation
composition function and diseases of the blood
 The average adult human 5 litter of blood.
 blood accounts for about 7 % of the body weight.
Blood:
 It is connective tissue a mixture of cellular component suspended in
fluid called plasma.
 Blood transports gases, nutrients, metabolic, wastes, blood cells,
immune cells, and hormones throughout the body
 The cellular component of blood consists of three primary cell types
 Erythrocytes (red blood cells [RBCs],
 Leukocytes (white blood cells [WBCs]), and
 Thrombocytes (platelets).
Red Blood cells
 Erythrocytes are flexible, a nuclear (lacking a nucleus), biconcave
disks Covered by a thin membrane through which oxygen (O2) and
carbon dioxide (CO2) pass freely.
 The production of erythrocytes is called erythropoiesis.
 The rate of erythrocyte production is regulated by erythropoietin, a
hormone released by the kidneys.
 The normal number of erythrocytes varies with age, gender and
altitude but is between 3.6 and 5.4 million/mm3.
 Erythrocytes circulate in the blood for about 120 days, after which the
spleen removes them.
White blood cell
 White blood cells are formed in the red bone marrow, lymphatic
tissue, spleen, lymph nodes, and thymus.
 The white blood cells are less numerous than the red blood cells and
larger than red blood cells.
 Compared to the red blood cells the white blood cells number only 1
white blood cell to 700 red blood cells.
 Leukocytes perform various protective functions such as engulfing
invading microorganisms and cellular debris, and manufacturing
antibodies the normal leukocyte count is between 5000 and 10,000
 An increased number of Leukocytes is called leukocytosis; a
decreased number is called leukopenia.
 The life span of leukocytes is only 1 to 2 days.
Platelets
 Platelets (thrombocytes) are disk like, non-nucleated cell Fragments
with a life span of approximately 7.5days.
 they are Manufactured in the redbone marrow.
 The number of platelets fall between the number of white blood cells
and red blood cells.
 The number of platelets can be between 250,000 and 350,000 in
normal volume of blood.
 The platelets get their name from their shape.
 They look like oval plate.
 Plasma
 About 55% of whole blood is blood plasma, a fluid that is the blood's
liquid medium, which by itself is straw-yellow in color.
 The blood plasma volume totals of 2.7–3.0 liters it consists of 90%
water and 10% proteins.
 Besides blood cells, plasma contains and transports proteins
(albumin, globulins, and fibrinogen), clotting factors such as
prothrombin, pigments, vitamins, glucose, lipids, electrolytes,
minerals, enzymes, and hormones.
Laboratory test Normal value Measures

Laboratory values related to red blood cells

Red blood cell

-4.2-5.4 Number of circulating RBCs
count
million/mm3 in one cubic millimeter
*Men
-3.6-5.0 (mm³) of blood
*Women
million/mm3
-1-1.5% of total Number of immature RBCs
Reticulocyte count
RBC in 1 mm3 of blood
Hemoglobin (Hgb)
Amount of hemoglobin in
*Men -14-16.5 g/dl
100 ml (1 dl) of blood
*Women 12-15g/dl
Hematocrit (HCT) Packed volume of RBCs in
*Men 40-50% 100 ml of blood, reported
*Women 37-47% as a percentage
*Mean corpuscular Average volume of
volume (MCV) individual RBCs
 85-100µm³ /cell
*Mean corpuscular Weight of the hemoglobin
31-35 g/dL
hemoglobin (MCH) in an average RBC
White blood cell
5.000-10.000/mm³
count
Laboratory values related to platelets and coagulation studies
150.000to300.000/ The number of circulating
Platelets
mm³ platelets in the blood.
Used to screen for
Bleeding time 2-9.5 minutes disorders caused by
platelet dysfunction
Evaluates the extrinsic
10-13 seconds
Prothrombin time clotting pathway;
(varies by
(PT or protime) prolonged in coumadin
laboratory)
therapy
INR (international Used to evaluate coumadin
2-3.5
normalized ration) therapy
Evaluate the intrinsic
Activated partial
clotting pathway;
thromboplastin 25-35 seconds
prolonged in heparin
time (APTT, PTT)
therapy

Evaluates conversion of
Thrombin time 24-35 seconds
fibrinogen to fibrin
Assessment of the patient with hematological disorders:
 Health history
 Physical examination
 Diagnostic study
Health History
Chief complaint
 Assess chief complaint quality and quantity, severity, and
location. it may be fever, fatigue, malaise, bleeding
 Assess patient signs and symptoms
 In case of anemia, the patient manifest fatigue, pallor,
weakness, bleeding disorders
 In case of bleeding, assess for bleeding disorders, and causes,
for instance liver disease, congenital coagulation disorders.
Assess demographic data.
 Obtain the patient’s biographic data. information about age,
sex, marital status, occupation, religion, race, and ethnic
background can provide important clues to risk factors.
 Family history:
 Some hematologic disorders are inherited.
 Ask about deceased family members, recording age at death
and cause.
 note any inheritable hematologic disorders and plot them on a
family genogram to determine the inheritance risk.
Past medical history:
 Assess for hematological disorders, ask whether there is a
history of anemia, any chronic diseases, cancer or kidney
disease, or HIV disease.
 Allergies
 Remember to look for allergies (including drug reactions) and
blood transfusion reaction
 Surgical history:
 Assess for any surgical operation because some GI operation
lead to anemia e.g., removal of portion of ileum effect on B12
absorption
 Medication
 Ask about past and current medications. There are some
medication leads to immune – suppression to bone marrow
 Social history:
 Ask him about alcohol intake, diet, sexual habits, and possible
drug abuse, all of which impair hematological function.
 Alcoholism can cause folic acid deficiency anemia
 Exposure to certain hazardous substances (such as benzene)
may cause bone marrow dysfunction, especially leukemia.
 Dietary habits:
 Hematological system depends on adequate intake of protein,
calories, and vitamins.
  Vital signs
 Vital signs can provide important clues.
 Frequent fevers can indicate a poorly functioning immune
system, hypotension possibly caused by septicemia or
hypovolemia, check respirations.
 The body’s difficulty in meeting its oxygen needs may cause
pronounced tachypnea.
Diagnostic tests
 A number of laboratory and diagnostic tests can be done to identify
disorders of the blood
 The complete blood count (CBC): is often performed as a
routine screening examination. The CBC includes the red blood
cell (RBC), the hemoglobin and hematocrit, the white blood cell
and platelet count
 Clotting studies or a coagulation profile are performed to
evaluate clotting and bleeding disorders, and often are ordered
prior to major surgeries (such as heart surgery, joint
replacements) these tests also are used to monitor thrombolytic
(fibrinolytic) and anticoagulant therapy
 Coombs’ test: is used to diagnose hemolytic anemias and
investigate transfusion reactions. The expected results are no
detected antibodies to RBCs (indirect coombs’) or no detected
RBC antigen – antibody complexes (direct coombs’)
 Hemoglobin electrophoresis: is performed when sickle cell
anemia or another genetically linked anemia is suspected. In
this test, blood is examined for the presence of hemoglobin S,
an abnormal form of the hemoglobin molecule.
  Tests of body iron stores are performed to evaluate iron
deficiency anemia
 Schilling’s test (vitaminB12 test): may be ordered to detect
pernicious anemia in clients with vitamin B12 deficiency. This
timed test evaluates the body’s ability to absorb vitamin B12 from
the GI tract. In this test, both an oral dose of radioactively tagged
vitamin B12 and an intramuscular vitamin B12 injection are
administered, followed by collection a 24 hour urine specimen.
the expected result is excretion of 10%or more of the
radioactively tagged vitamin B 12 within 24 hours.
 Biopsy: when a hematologic or lymphatic malignancy is
suspected, bone marrow or tissue from a lymph node is
microscopically examined for the presence of abnormal cells.
 Bone marrow aspiration: in many hematological disorders, it
is necessary to aspirate and analyze bone marrow to establish
the diagnosis. Once obtained, the bone marrow is analyzed for
the different types of cells it contains. Normally, there are up to
15 different types of cells present. The number, appearance,
and development of the various blood cell types are analyzed to
diagnose hemolytic blood disorders, tumors, leukemias, and in
some cases, infectious diseases .in adults’ bone marrow may
be obtained from the posterior iliac crest, anterior iliac crest, or
sternum.
Nursing role in Bone marrow examination
 Before performing a procedure, the nurse should ensure the correct
identification of the patient
  Obtain informed consent for bone marrow aspiration and biopsy
 Prepare equipment
 minimize discomfort during the procedure
 Patient may need analgesia or sedation
 Place the patient on a cardiac monitor. Rationale: This allows for
assessment of patient status during procedure
 Assist the patient to an appropriate position depending on the
patient’s comfort and the preference of the physician,
 Ensure site markings have been made where appropriate.
(Performed on the back of the hipbone, or posterior iliac crest.)
During and after procedure
 Assess vital signs, oxygenation, level of consciousness, and cardiac
rhythm during the procedure and until the patient is completely
recovered from sedation medications.
 Assess the site at regular intervals with consideration of patient
specific risk factors for bleeding.
 Monitors for signs and symptoms of complications. (Bleeding -
Hematoma - Pain –Infection)
 keep pressure dressing clean, dry, and in place for 24 hours after the
procedure.
 Advise the patient and family to apply a wrapped ice bag to the site
over clothing This will also reduce the risk of bleeding. The ice should
never be applied directly to the skin.
 Advise against (non-steroidal anti-inflammatory drugs or aspirin) for
24 hours after biopsy.
  instruct the patient and family on the appropriate time frame to restart
anticoagulation if being held.
hematological disorders
A. Red blood cell disorders
 Disorders affecting the red blood cells affect the body’s ability to carry
oxygen to the tissues.
 the client often experiences manifestations such as fatigue and
activity intolerance.
 in general, when there are too few red blood cells, the disorder is
known as anemia.
 An excess of red blood cells is known as polycythemia or
erythrocytosis.
1. Anemia
Definition:
 Anemia is a deficiency in the number of red blood cells (RBCS) or
erythrocytes, the quantity of hemoglobin (HB)and/ or the volume of
packed red cells (hematocrit).
 Anemia can be caused by blood loss, impaired production of
erythrocytes, or increased destruction of erythrocytes.
Classification of Anemias
 Anemia may be classified in several ways.
 The physiologic approaches to determine whether the deficiency in
RBCs is caused by a defect in their production (hyperproliferative
anemia), by their destruction (hemolytic anemia), or by their loss
(bleeding).
 In the hyperproliferative anemia, RBCs usually survive normally, but
the marrow cannot produce adequate numbers of these cells.
 The decreased production is reflected in a low reticulocyte count.
 Inadequate production of RBCs may result from marrow damage due
to medications or chemicals (e.g., chloramphenicol, benzene) or from
a lack of factors necessary for RBC formation (e.g., iron, vitamin B12,
folic acid, erythropoietin.
  Hemolytic anemia stem from premature destruction of RBCs, which
results in a liberation of hemoglobin from the RBC into the plasma.
 The increased RBC destruction results in tissue hypoxia, which in
turn stimulates erythropoietin production.
 This increased production is reflected in an increased reticulocyte
count, as the bone marrow responds to the loss of RBCs.
 The released hemoglobin is converted in large part to bilirubin;
therefore, the bilirubin concentration rises.
 Hemolysis can result from an abnormality within the RBC itself (e.g.,
sickle cell anemia, glucose-6-phosphatedehydrogenase [G-6-PD]
deficiency) or within the plasma (e.g., immune hemolytic anemias), or
from direct injury to the RBC within the circulation (e.g., hemolysis
caused by mechanical heart valve).
Pathophysiology of anemia:
 Anemia reduces the oxygen-carrying of the blood, leading to tissue
hypoxia. As tissue oxygenation decreases, the body attempts to
restore adequate oxygen delivery. Blood is redistributed to vital
organs, causing the following clinical manifestation.
Clinical manifestation of anemia
 Pallor of the skin, mucous membranes, nail beds and conjunctiva.
Tissue hypoxia may cause angina,
 fatigue, dyspnea on exertion, and night cramps.
 The kidneys release increased amounts of erythropoietin, which
stimulates the bone marrow, causing bone pain. poor oxygen delivery
to the brain can cause headache, dizziness, and dim vision
 The intensity of manifestations varies depending on severity of
anemia.
 The severity of anemia can be determined by the Hb level.
1. Mild state of anemia (Hb 10 to 14 g/dl) may exist without causing
symptoms.
 If symptoms developed due to compensatory response to heavy
exercise.
2. moderate state (Hb 6 to 10 g/dl)
 Palpitation and dyspnea with rest as well as activity.
3. severe anemia (Hb less than 6 g/dl):
 skin  pallor, jaundice.
 Eyes  blurred vision retinal hemorrhage.
 Cardiovascular  palpitation, tachycardia increase pulse, CHF,
and MI.
 neurological  headache, vertigo, irritability, depression.
 Gastrointestinal  anorexia, hepatomegaly.
Nursing process:
The patient with Anemia
 Assessment
 The health history and physical examination provide important data
about the type of anemia involved, the extent and type of symptoms it
produces, and the impact of those symptoms on the patient’s life.
Diagnosis
 NURSING DIAGNOSES
Based on the assessment data, major nursing diagnoses for the
anemic patient may include:
 Activity intolerance related to weakness, fatigue, and general
malaise.
 Imbalanced nutrition, less than body requirements, related to
inadequate intake of essential nutrients.
 Ineffective tissue perfusion related to inadequate blood volume
or hematocrit.
 Noncompliance with prescribed therapy.
 Nursing Interventions
MANAGING FATIGUE
 nursing interventions can focus on assisting the patient to prioritize
activities and to establish a balance between activity and rest that is
realistic and feasible from the patient’s perspective. Patients
 with chronic anemia need to maintain some physical activity and
exercise to prevent the deconditioning that results from inactivity
 Determine the level and cause of fatigue.
  Monitor the patient’s blood indices such as hematocrit, hemoglobin,
NRBC counts, and special tests such as reticulocyte counts
 Evaluate the patient’s ability to carry out ADLs and the capacity to do
his/her usual routine tasks. (Fatigue brought on by anemia manifests
itself in various ways, affecting cognitive capacity and social and
emotional well-being. However, the most typical manifestation is
activity intolerance. Usually, fatigue hinders the patient’s capacity to
participate and fulfill their societal and familial responsibilities (e.g.,
working away from home), which makes it difficult to perform certain
tasks.)
MAINTAINING ADEQUATE NUTRITION
 maintaining adequate nutrition. A healthy diet should be encouraged.
Because alcohol interferes with the utilization of essential
 nutrients, the nurse should advise the patient to avoid alcoholic
beverages or to limit their intake and should provide the rationale for
this recommendation.
 Dietary teaching sessions should be individualized, including cultural
aspects related to food preferences and food preparation. The
involvement of family members enhances compliance with dietary
recommendations.
 Dietary supplements (e.g., vitamins, iron, folate, protein) may be
prescribed as well.
 Equally important, the patient and family must understand the role of
nutritional supplements in the proper context, because many forms of
anemia are not the result of a nutritional deficiency. In such cases,
excessive intake of nutritional supplements will not improve the
 anemia. A potential problem in individuals with chronic transfusion
requirements occurs with the indiscriminate use of iron.
MAINTAINING ADEQUATE PERFUSION
 Patients with acute blood loss or severe hemolysis may have
decreased tissue perfusion from decreased blood volume or reduced
circulating RBCs (decreased hematocrit). Lost volume is replaced
with transfusions or intravenous fluids, based on the symptoms and
the laboratory findings. Supplemental oxygen may be necessary, but
it is rarely needed on a long-term basis unless there is underlying
severe cardiac or pulmonary disease as well. The nurse monitors
vital signs closely; other medications, such as antihypertensive
agents, may need to be adjusted or withheld.
PROMOTING COMPLIANCE WITH PRESCRIBED THERAPY
 For patients with anemia, medications or nutritional supplements are
often prescribed to alleviate or correct the condition.
 These patients need to understand the purpose of the medication,
how to take the medication and over what time period, and how to
manage any side effects of therapy.
 To enhance compliance, the nurse can assist patients in developing
ways to incorporate the therapeutic plan into their lives, rather than
merely giving the patient a list of instructions.
 For example, many patients have difficulty taking iron supplements
because of related gastrointestinal effects.
 Rather than seeking assistance from a health care provider in
managing the problem, some of these patients simply stop taking the
iron.
  Abruptly stopping some medications can have serious
consequences, as in the case of high-dose corticosteroids to manage
hemolytic anemia.
 Some medications, such as growth factors, are extremely expensive.
Patients receiving these medications may need assistance with
obtaining needed insurance coverage or with exploring alternatives
for obtaining these medications.
MONITORING AND MANAGING POTENTIAL COMPLICATIONS
 A significant complication of anemia is heart failure from chronic
diminished blood volume and the heart’s compensatory effort to
increase cardiac output.
 Patients with anemia should be assessed for signs and symptoms of
heart failure.
 A serial record of body weights can be more useful than a record of
dietary intake and output because the intake and output
measurements may not be accurate.
 In the case of fluid retention resulting from congestive heart failure,
diuretics may be required.
 In megaloblastic forms of anemia, the significant potential
complications are neurologic.
 A neurologic assessment should be performed for patients with
known or suspected megaloblastic anemia.
 Patients may initially complain of paresthesia in their lower
extremities.
 These paresthesia are usually manifested as numbness and tingling
on the bottom of the foot, and they gradually progress.
  As the anemia progresses and damage to the spinal cord occurs,
other signs become apparent.
 Position and vibration sense may be diminished; difficulty maintaining
balance is not uncommon, and some patients have gait disturbances
as well.
 Initially mild but gradually progressive confusion may develop.
EXPECTED PATIENT OUTCOMES
Expected patient outcomes may include:
 Tolerates activity at a safe and acceptable level
 Attains and maintains adequate nutrition
 Maintains adequate perfusion
 Has vital signs within baseline for patient
 Has pulse oximetry (arterial oxygenation) value within normal limits
 Absence of complications
1. Iron deficiency anemia:
Definition
 Iron deficiency anemia, one of the most common chronic hematologic
disorders, is found in 30 %of the world’s population.
 is caused by an inadequate supply of iron for RBC formation.
Pathophysiology
 Iron deficiency anemia develops when iron is insufficient to produce
hemoglobin.
 Iron is present in RBCs as heme in HB, hem accounts for 2/3 of the
body iron.
 When iron deficiency develops, iron stores in the body are depleted
followed by reduced hemoglobin.
  This result to smaller and fewer RBCs which lead to anemia.
 Daily need:10mg/day, only 1mg is absorbed
Causes of iron deficiency anemia
 Inadequate dietary intake of iron, as in prolonged un supplemented
breast –or bottle-feeding of infants
 Iron malabsorption, as in chronic diarrhea, partial or total gastrectomy
 Pregnancy, in which the mother’s iron supply is diverted to the fetus
for erythropoiesis
 Blood loss secondary to drug- induced GI bleeding (from anticoagulants,
aspirin, steroids) or due to heavy menses, hemorrhage from trauma.
Clinical Manifestations of Iron Deficiency Anemia
 In the early course of iron deficiency anemia, the patient may be free
of symptoms
 pallor is the most common finding, and glossitis (inflammation of
tongue) is the second most common; another finding is cheilitis
(inflammation of lips)
 In addition, the patient may report headache, fatigue, weakness, and
dyspnea, jaundice, brittle nails, back pain, joint pain. abdominal pain
all of which are caused by lack of iron in the tissues.
Diagnosis
 Blood study: ↓ hemoglobin, hematocrit, and serum iron levels.
 bone marrow studies reveal depleted or absent iron stores
 other laboratory and diagnostic tests (e.g., stool examination for
occult blood) reveal the source of blood loss
Medical management
 Treatment aims at determining the cause and, when possible,
eliminating it.
 Iron Replacement therapy. (Oral – IV – IM)
 Before parenteral
 administration of a full dose, a small test dose should be
administered to avoid the risk of anaphylaxis with either
intravenous or intramuscular injections.
 Emergency medications (e.g., epinephrine) should be close at
hand.
 If no signs of allergic reaction have occurred after 30 minutes,
the remaining dose of iron may be administered.
 Several doses are required to replenish the patient’s iron stores
 In severe cases, a blood transfusion is necessary.
2. Megaloblastic Anemia
 Anemia caused by deficiencies of vitamin B12 or folic acid,
characterized by the RBCs, large in size and called megaloblastic
RBCs.
 Vitamin B12 deficiency anemia.
 Folic acid deficiency anemia.
1. Vitamin B12 Deficiency (cobalamin):
 Anemia resulting from a cobalamin (vitamin B12) deficiency is a type
of megaloblastic anemia caused by impaired DNA synthesis.
 It is an autoimmune disorders characterized by absence of intrinsic
factor IF (specific type of protein) that is secreted by gastric mucosa
leading to mal absorption of vitamin B12.
Causes
 vitamin B12 deficiency can occur in patients who have had GI surgery,
such as gastrectomy; patients who have had a small bowel resection
involving the ileum; and patients with Crohn’s disease, ileitis,
diverticula of the small intestine, and /or chronic atrophic gastritis
Pathophysiology of Pernicious Anemia
 vitamin B12 deficiency results from the loss of IF-secreting gastric
mucosal surface or impaired absorption of cobalamin in the distal
ileum. Because an acid environment in the stomach is required for
the secretion of IF, cobalamin deficiency is also found in long – term
users of H2 – histamine receptor blockers.
 Pernicious anemia is caused by an absence of IF, either from gastric
mucosal atrophy or autoimmune destruction of parietal cell.
destruction of the parietal cell prevents both IF
Signs and Symptoms of Vit B12 deficiency
 In addition to the usual symptoms of anemia, some clients with
pernicious anemia develop
 Stomatitis (inflammation of the mouth).
 Glossitis (inflammation of the tongue).
 Digestive disturbances and diarrhea.
 Dyspnea occurs with minimal exertion.
 Jaundice, irritability, confusion, and depression are present when the
disease is severed.
 Numbness in the arms and legs and ataxia are common signs of
neurologic involvement
Diagnosis of PA:
 Full blood count
 B12 level
 Intrinsic factor auto antibodies and partial cell auto antibodies
 A schilling test can be used to assess parietal cell function and is
diagnostic of pernicious anemia if orally administered radioactive
cobalamin is absorbed following the parenteral administration of IF.
Medical Management:
 Administer Vit B12 IM in dose adequate to control the disease.
 Therapy must continue for life long.
 No toxic effects from use of vitamin B12.
Medical Management of Pernicious Anemia
 Immediate Therapy
 Parental administration of vitamin B12 daily for 2 weeks, then
weekly until HCT return to normal.
 Lifelong Therapy
 Monthly injection for life is required
 An intranasal form of medication is available as a nasal gel that
is self-applied weekly.
 Injection usually has quick response within 72 hours,
reticulocytes begin to increase, RBCs count rises significantly.
 No toxic effects from use of vitamin B12.
2. Folic acid deficiency anemia:
 Folic acid is required for DNA synthesis leading to red blood cell
(RBC) (erythrocyte) formation and maturation.
  a deficiency of Folic acid results in a megaloblastic anemia with large,
immature RBCs
Causes:
 poor nutrition, especially a lack of leafy green vegetables, liver, citrus
fruits, yeast, dried beans, nuts, and grains
 malabsorption syndromes, particular small bowel disorders
 drugs that impede absorption and use of folic (e.g., methotrexate),
anti-seizure medications (e. g, Phenobarbital, phenytoin.
 Alcohol abuse and anorexia
 Hemodialysis treatments, since folic acid is lost during dialysis
Clinical manifestation
 The disease develops insidiously, and the patient’s symptoms may be
attributed to other coexisting problems such as cirrhosis or
esophageal varices.
 Gastrointestinal (GI) disturbance include dyspepsia and a smooth,
beefy red tongue
 The clinical manifestation similar to vit B12 deficiency.
 The absence of neurologic problem is an important diagnostic finding.
Laboratory finding
 blood test results reveal low hemoglobin and
 hematocrit level, macrocytosis,
 decrease reticulocyte count, increased abnormal platelet count and
serum folate levels below 4 mg /ml
Management of Folic acid deficiency
 Medical management consists primarily of folic acid supplements and
elimination of contributing causes.
  The patient may Take supplements orally (1 to 5 mg /day) or
parenterally (for patients who are severely ill, have mal absorption, or
can’t take oral medication).
 Many patients respond favorably to a well- balanced diet that includes
foods high in folic acid.
Nursing role
 Assessment:
 The patient’s history may reveal severe, progressive fatigue,
the hallmark of folic acid deficiency. Associated finding include
shortness of breath, palpitations, diarrhea, nausea, anorexia,
headaches, forgetfulness, and irritability. weakness and light-
headedness
 Provide well balanced diet, including foods high in folate, such as
dark green leafy vegetables, organ, meats, eggs, milk, bananas, dry
beans, and whole grain breads
 Monitor fluid and electrolyte balance, particularly in the patient who
has severe diarrhea and is receiving parenteral fluid replacement
therapy.
 If the patient have severe anemia, plan activities, rest periods, and
diagnostic tests to conserve his energy.
 Help the patient maintain body alignment and mobility; begin with
gentle range of motion exercises, as tolerated, and progress to out of
bed activity.
 monitor the patient’s complete blood count, platelet count, and serum
folate level as ordered.
 3. Hemolytic anemia
Definition:
 It's a chronic premature destruction of erythrocytes occurring as such
a rate the bone marrow is unable to compensate for loss of cells, it
can be acquired, idiopathic or hereditary.
Causes:
 Inherited Hemolytic Anemia
 In inherited hemolytic anemia, the genes that control how red
blood cells are made are faulty. The patient can receive a faulty
red blood cell gene from one or both of parents.
 (Sickle cell anemia _ Thalassemia.
 Acquired Hemolytic Anemia
 In acquired hemolytic anemia, the body makes normal red
blood cells. However, some disease, condition, or factor
destroys the cells too early. Examples include immune
disorders, infections, and reactions to medicines or blood
transfusion.
 EX. Autoimmune hemolytic anemia (AIHA)
Clinical Manifestations
 The patient who has hemolytic anemia manifested by the same signs
and symptoms of anemia as follows.
 Shortness of breath; dizziness.
 Headache.
 3-Coldness in the patient hands or feet.
 Pale skin, gums, and nail beds.
 Chest pain, arrhythmias.
  Heart murmur, Heart Failure
 Jaundice
 Pain in the upper abdomen; due to gallstones or an enlarged
spleen.
 High levels of bilirubin and cholesterol (from the breakdown of
red blood cells) can form into stones in the gallbladder.
Diagnostic Studies of Hemolytic Anemia
 CBC
 Reticulocyte count: People who have hemolytic anemia usually have
high reticulocyte counts because their bone marrow is working hard
to replace the destroyed red blood cells.
 Blood Smear.
 Coombs' test.
 This test can show whether the body is making antibodies
(proteins) to destroy RBCs.
 Bilirubin:
 Hemoglobin is broken down into a compound called bilirubin
and hem. High levels of bilirubin in the blood may be a sign of
hemolytic anemia.
Management of Hemolytic Anemia
Goals of Treatment
 The goals of treating hemolytic anemia include:
 Reducing or stopping the destruction of red blood cells.
 Increasing the red blood cell count to an acceptable level.
 Treating the underlying cause of the condition.
1. Blood Transfusions
 Blood transfusions are used to treat severe or life-threatening
hemolytic anemia.
 Transfusions require careful matching of donated blood with the
recipient's blood.
2. Medicines
 Medicines can improve some types of hemolytic anemia,
especially autoimmune hemolytic anemia (AIHA).
 Corticosteroid medicines, such as prednisone, are used to limit
or prevent immune system from making antibodies against red
blood cells.
 If the patient doesn’t respond to corticosteroids, the physician
may prescribe other medicines to suppress immune system
3. Blood and Marrow Stem Cell Transplant
 In some types of hemolytic anemia, such as, the bone marrow
doesn't make enough healthy red blood cells. The red blood cells it
does make may be destroyed before their normal lifespan is over.
Bone marrow stem cell transplants may be used to treat these types
of hemolytic anemia.
4. Plasmapheresis
 It is a procedure that removes antibodies that formed against RBCs
from the blood. For this procedure, blood is taken from the body using
a needle inserted into a vein. The plasma, which contains the
antibodies, is separated from the rest of the blood. Then, plasma from
a donor and the rest of the blood is put back in the body.
 Nursing Care for Patient undergoing Plasmapheresis
Before Procedure
 Verify plasmapheresis orders/consent.
 Assist in gathering the supplies for vascular access.
 Ensure that appropriate replacement.
 Assist with set up and priming of the Ensures safe and proper
assembly and plasmapheresis circuit as needed.
 Tape and secure all connections.
During and after Procedure
 Monitor the patient throughout and after patients can experience
complications hypotension, hypothermia, blood leak, air embolism.
 Monitor vital signs.
 Monitor Plasmapheresis circuit.
 Monitor laboratory values as ordered may require intervention in case
of hemolysis, thrombocytopenia.
 Monitor serum electrotypes
4. Sickle cell anemia
 It is a sever hemolytic anemia characterized by presence of abnormal
shape of HB in RBCs. (Presence of hemoglobin S instead of normal
hemoglobin A)
Pathophysiology of Sickle Cell
 It is a serious disease in which RBCs makes “Sickle-shaped” means
like "C." Sickle-shaped cells don’t move easily through the blood
vessels. They are stiff and sticky and tend to form clumps and get
stuck in the blood vessels. Sickle cells block blood flow in the blood
vessels that lead to decrease blood flow to body organs which leads
 to limbs and organs damage, and this blockage lead to sickle cell
crisis.
 If the person inherits only one gene, he or she carries sickle cell trait.
 The hemoglobin of those who have sickle cell trait is about 40%
affected.
 Consequently, those people are at less risk for developing signs and
symptoms than those who have sickle cell disease.
Clinical Manifestations
 Sickle cell anemia is present at birth, but many infants don't show any
signs until after 4 months of age.
 The most common signs and symptoms are linked to anemia, except
sickle episode.
Signs and Symptoms of Sickle Episode
 Sudden pain throughout the body, this pain is called a "sickle cell
crisis." often affect the bones, lungs, abdomen, and joints.
 The pain from sickle cell crises can be acute or chronic, but acute
pain is more common. Acute pain is sudden and can range from mild
to very severe. The pain usually lasts from hours to a few days.
Chronic pain often lasts for weeks to months. Chronic pain can be
hard to bear and mentally draining.
1. Acute Episodes of sickle cell:
 Pain usually in back, chest or extremities,
 may be localized or generalized.
 Fever low grade, 1-2 days after onset of pain.
 Evidence of accelerated rate of erythrocytes destruction is manifested
by jaundice (caused by hyper bilirubinemia).
  Secondary consequence include gallstone.
 ↑ the predisposition to infection (occur when the spleen becomes
dysfunctional.
2. Chronic Hemolytic Anemia: -
 Chronic leg ulcers develop from blockage of the small blood vessel of
the legs.
 The reduced blood flow during sickle cell crisis lead to: -
 Renal problems is renal insufficiency from repeated infraction.
 Severe pain, due occlusion of the small blood vessel with abnormal
hemoglobin.
 Swelling of one more joints are common.
 Other symptoms depend on the blood vessel involved.
 Enlarged facial and skull bones
 Leg ulcers, usually caused by trauma
 Splenomegaly
 Cardiomegaly
 Tachycardia, flow murmurs.
 Growth retardation.
 Delayed puberty
 In adulthood characteristics spider body habitus (e.g.,
elongated extremities, narrow shoulders and hips, barrel chest,
curved spine elongated skull).
The Diagnostic Findings:
 The patient with sickle cell trait usually has a normal hemoglobin
level, a normal hematocrit, and a normal blood smear. In contrast, the
patient with sickle cell anemia has a low hematocrit and sickled cells
 on the smear. The diagnosis is confirmed by hemoglobin
electrophoresis
 Sickle cell screening test, called sickledex test that determine the
presence of abnormal (HbS).
Management of Sickle Cell Anemia
 Bone marrow transplantation, cure sickle cell disease in a few cases
 Hydroxy urea
 If the patient has severe sickle cell anemia, may need a
medicine called hydroxyurea. This medicine helps reduce the
number of painful crises when they occur. Hydroxyurea can
cause serious side effects, including an increased risk for
dangerous infections
 Blood transfusion:
 Regular blood transfusion decrease the risk for stroke and other
complications of infarction. may cause complication as iron
overload treat with erthrocytopheresis
 O2 therapy, to relieve hypoxia.
 Antibiotics.
 Folic acid is prescribed to facilitate the replacement of hemolyzed
RBCs.
 Analgesic, to treat the severe pain that's associated with sickle cell
crisis.
 Bed rest.
 IV fluids for hydration.
NURSING PROCESS:
 Patients in sickle cell crisis should be assessed for factors that could
have precipitated the crisis,
 Pain levels should always be monitored; The quality of the pain (e.g.,
sharp, dull, burning), the frequency of the pain factors that aggravate
or alleviate the pain.
 Asses for signs of dehydration
 Assess symptoms of infection
 Assessment of all body systems is necessary. Because the sickling
process can interrupt circulation in any tissue or organ, (All joint areas
are carefully examined for pain and swelling. The abdomen is
assessed for pain and tenderness because of the possibility of
splenic infarction. The respiratory system must be assessed
carefully,)
Diagnosis
 NURSING DIAGNOSES
 Based on the assessment data, major nursing diagnoses for the
patient with sickle cell crisis may include:
 Acute pain related to tissue hypoxia due to agglutination of
sickled cells within blood vessels
 Risk for infection
 Risk for powerlessness related to illness-induced helplessness
 Deficient knowledge regarding sickle crisis prevention
  Planning and Goals
 The major goals for the patient are relief of pain, decreased
incidence of crisis, enhanced sense of self-esteem and power,
and absence of complications.
 Nursing Interventions
Managing pain
 Assess pain level by pain scale
 use of analgesics, which are valuable
 Any joint that is acutely swollen should be supported and elevated
until the swelling diminishes.
 Relaxation techniques
 breathing exercises
 distraction are helpful for some patients.
Preventing and managing infection
 Nursing care focuses on monitoring the patient for signs and
symptoms of infection.
 Prescribed antibiotics should be initiated promptly, and the patient
should be assessed for signs of dehydration.
 If the patient is to take prescribed oral antibiotics at home, he or she
must understand the need to complete the entire course of antibiotic
therapy and must be able to identify a feasible administration
schedule.
Promoting Coping skills
 This illness, because of its acute exacerbations that often result in
chronic health problems, frequently leaves the patient feeling
powerless and with decreased self-esteem.
  These feelings can be exacerbated by inadequate pain management.
 The patient’s ability to use normal coping resources of physical
strength, psychological stamina, and positive self-esteem is
dramatically diminished.
 Enhancing pain management can be extremely useful in establishing
a therapeutic relationship based on mutual trust.
 Nursing care that focuses on the patient’s strengths rather than
deficits can enhance effective coping skills.
 Providing the patient with opportunities to make decisions about daily
care may increase the patient’s feeling of control.
Provide health education and Minimizing deficit knowledge
 Patients with sickle cell anemia benefit from understanding disease,
signs, potential complication, and what situations can precipitate a
sickle cell crisis and the steps they can take to prevent or diminish
such crises.
 Keeping warm and maintaining adequate hydration can be very
effective in diminishing the occurrence and severity of attacks.
 Avoiding stressful situations
Monitoring and managing potential complications
 Management measures for many of the potential complications were
delineated in previous sections. Other measures follow.
 Leg Ulcers
 Leg ulcers require careful management and protection from
trauma and contamination. Referral to a wound care specialist
may facilitate healing and assist with prevention. If leg ulcers
 fail to heal, skin grafting may be necessary. Scrupulous aseptic
technique is warranted to prevent nosocomial infections.
Priapism Leading to Impotence
 Male patients may develop sudden, painful episodes of priapism
(persistent penile erection). The patient is taught to empty his bladder
at the onset of the attack, exercise, and take a warm bath. If an
episode persists longer than 3 hours, medical attention is
recommended. Repeated episodes may lead to extensive vascular
thrombosis, resulting in impotence.
Chronic Pain and Substance Abuse
 Many patients have considerable difficulty coping with chronic pain
and repeated episodes of sickle crisis. Those who feel they have little
control over their health and the physical complications that result
from this illness may find it difficult to understand the importance of
complying with a prescribed treatment plan. Being nonjudgmental
and actively seeking involvement from the patient in establishing a
treatment plan are useful strategies.
 Some patients with sickle cell anemia develop problems with
substance abuse. For many, this abuse results from inadequate
management of acute pain during episodes of crisis. Some clinicians
suggest that abuse may result from prescribing inadequate amounts
of opioid analgesics for an inadequate time. The patient’s pain may
never be adequately relieved
Continuing Care
 The illness trajectory of sickle cell anemia is highly varied, with
unpredictable episodes of complications and crises. Care is often
 provided on an emergency basis, especially for some patients with
pain management problems (see previous section). Nurses in all
settings used by this patient population need to communicate
regularly with each other. Patients need to learn which parameters
are important for them to monitor and how to monitor them.
Parameters should also be given as to when to seek urgent care.
Evaluation
 Expected patient outcomes may include:
 Control of pain
 Acute pain is controlled with analgesics
 Expresses improved sense of control
 Participates in goal setting and in planning and implementing
 daily activities
 Participates in decisions about care
 Increases knowledge about disease process
 Absence of complications
2. Polycythemia
Definition:
 Polycythemia (also called erythrocytosis) is an abnormally high red
blood cell count with high hematocrit. when the hematocrit is greater
than to more than 55% in males, more than 50% in females viscous
or “sticky”.
Pathophysiology:
 Polycythemia vera, or primary polycythemia, is a proliferative disorder
in which the myeloid stem cells seem to have escaped normal control
mechanisms.
  The bone marrow is hypercellular, and the RBC, WBC, and platelet
counts in the peripheral blood are elevated.
 However, the RBC elevation is predominant‫ الغالب‬the hematocrit
 can exceed 60%.
 This phase can last for an extended period (10 years or longer).
 The spleen resumes its embryonic function of hematopoiesis and
enlarges.
 Over time, the bone marrow may become fibrotic, with a resultant
inability to produce as many cells as possible.
Causes of Polycythemia Vera
 Primary PV is due to genetic cause.
 Secondary PV due to:
 Lack of oxygen over a long period.
 Smoking.
 Long hours at high altitudes
Assessment finding
 Signs and symptoms
 The face and lips are reddish- purple
 Fatigue, weakness, headache, exertional dyspnea, and
dizziness are common
 Excessive bleeding after minor injuries, perhaps because of the
‫ احتقان‬engorgement of the capillaries and veins, occurs
 Hemorrhoids develop
 Hepatomegaly
 Gout
 Retinal veins thrombosis
  Splenomegaly
 The joints become swollen and painful because of elevated uric
acid levels
 Diagnostic finding:
 The blood cell count, especially erythrocytes, is elevated, with a
similar rise in hemoglobin and hematocrit levels. The platelet
and WBC counts are increased.
 Levels of serum potassium and uric acid are above normal.
 Platelet count above 400,000/µl(thrombocytosis)
 White blood cell count above 10,000/µl in adult (leukocytosis)
Medical management:
 Treatment involves measures to reduce the volume of circulating
blood, lessen its viscosity, and curb the excessive production of
erythrocytes.
 Blood donation
 A phlebotomy (opening a vein to withdraw blood) “a needle is inserted
into the vein, and the blood flows through an airtight tube into a sterile
container or bag” is done several times a week;500 ml of blood is
removed each time.
 Anticoagulants are prescribed to reduce the potential for forming
clots.
 Antihistamines are not particularly effective in controlling itching 5- 5
 (Zyloprim) is used to prevent gouty attacks.
 Radiation therapy can be used to decrease the production of
erythrocytes in the bone marrow.
  Hydroxyurea is a medicine generally used to treat cancer. This
medicine can reduce the number of red blood cells and platelets in
the blood. As a result, can be used to suppress marrow function but
they may increase the risk for leukemia.
 Interferon-alpha is a substance that the body normally produces. It
also can be used to treat PV. Interferon-alpha can prompt the
immune system to fight bone marrow cells that are making too many
red blood cells. As a result, this treatment can help lower the number
of red blood cells in the body and maintain blood flow and blood
thickness that's close to normal.
SECONDARY POLYCYTHEMIA
 Secondary polycythemia is caused by excessive production of
erythropoietin.
 This may occur in response to a reduced amount of oxygen, which
acts as a hypoxic stimulus, as in cigarette smoking, chronic
obstructive pulmonary disease, or cyanotic heart disease, or high
altitude.
 Secondary polycythemia can also occur from neoplasms
 (e.g., renal cell carcinoma) that stimulate erythropoietin
 production.
Medical Management
 Management of secondary polycythemia may not be necessary.
 when it is, it involves treating the primary problem. If the cause
cannot be corrected (e.g., by treating the renal cell carcinoma or
improving pulmonary function), therapeutic phlebotomy may be
 necessary in symptomatic patients to reduce blood viscosity and
volume.
Nursing Instructions for Patient with Polycythemia Vera
 Instruct patient to drink 3 ml fluids /day to promote venous return.
 avoid iron supplements,
 Advise patient to avoid crossing the legs at the knee and avoid
wearing tight clothes.
 Encourage the patient to be physically active.
 Teach patient how to perform isometric exercises.
 Instruct patient to wear thromboembolic stocking during waking hours
to improve venous return.
 Instruct patient if chest pain developed to notify physician and keep
rest immediately.
 Maintain ROM exercises and ambulation.
 Instruct to immediately report symptoms of thrombosis such as pallor
or pain in an extremity, chest or abdominal pain, and any abnormal
bleeding.
B. White blood cell disorders
1. Leukopenia,
 a condition in which there are fewer WBCs than normal, results from
neutropenia (diminished neutrophils) or lymphopenia (diminished
lymphocytes).
 Even if other types of WBCs (e.g., monocytes, basophils) are
diminished, their numbers are too few to reduce the total WBC count
significantly
 2. Lymphopenia
 lymphocytopenia refers to a lack of lymphocytes in the bloodstream.
 The usual range of blood lymphocyte levels differs for adults and
children.
 For adults lymphocytopenia occurs when the total number of
lymphocytes per microliter of blood falls below 1500.
 For children, lymphocytopenia: - occurs when this number falls below
3,000.
Causes
 Most of the time, lymphocytopenia is a condition that people acquire.
However, it can sometimes be present from birth.
 Some factors that can lead to acquired lymphocytopenia include:
 fasting or malnutrition
 severe physical stress
 cancer treatments, such as radiation therapy, and chemotherapy
 use of steroids
 autoimmune diseases, such as rheumatoid arthritis or lupus
 blood disorders, such as aplastic anemia
 infectious diseases, such as HIV, tuberculosis, and viral hepatitis
3. NEUTROPENIA
 Neutropenia (neutrophils less than 2000/mm3) results from
decreased production of neutrophils or increased destruction of these
cells Neutrophils are essential in preventing and limiting bacterial
infection.
  A patient with neutropenia is at increased risk for infection, The risk
for infection is based not only on the severity of the neutropenia (low
neutrophil count), but also on the duration of the neutropenia.
 The actual number of neutrophils, known as the absolute neutrophil
count (ANC), is determined by a simple mathematical calculation
using data obtained from the CBC and differential test (Chart The risk
of infection increases proportionately with the decrease in neutrophil
count.
 The risk is significant when the ANC is less than 1000, high when it is
less than 500, and is almost certain when it is less than 100.
Total WBCS count X (neutropenia %+ bands)
 The risk 100

Causes of Neutropenia
 Decreased Production of Neutrophils
 Aplastic anemia, due to medications or toxins
 Metastatic cancer, lymphoma, leukemia
 Myelodys plastic syndromes
 Chemotherapy
 Radiation therapy
 Ineffective Granulocytopoiesis
 Megaloblastic anemia
 Increased Destruction of Neutrophils
 Hypersplenism
 Medication-induced.
 Immunologic disease (e.g., systemic lupus erythematosus [SLE])
 Viral disease (e.g., infectious hepatitis, mononucleosis)
  Bacterial infections
 Formation of antibody to medication, leading to a rapid decrease in
neutrophils.
Nursing management
 Nurses in all settings have a crucial role in assessing the severity of
neutropenia and in preventing and managing infectious complications
 Assessment
Patient
 Assess the following areas thoroughly every shift or visit (with spot
checks throughout shift if hospitalized) and notify physician of any
signs of infection or worsening of status:
Skin:
 Check for tenderness, edema, breaks in skin integrity, moisture,
drainage, lesions (especially under breasts, axillae, groin, skin folds,
bony prominences, perineum); check all puncture sites (e.g.,
intravenous sites) for signs and symptoms of inflammation/infection.
Oral mucosa:
 Check for moisture, lesions, color (check palate, tongue, buccal
mucosa, gums, lips, oropharynx).
Respiratory:
 Check for presence of cough, sore throat; auscultate breath sounds.
Gastrointestinal:
 Check for abdominal discomfort/distention, nausea, change in bowel
pattern; auscultate bowel sounds.
Genitourinary:
  Check for dysuria, urgency, frequency; check urine for color, clarity,
odor.
Neurologic:
 Check for complaints of headache, neck stiffness, visual
disturbances; assess level of consciousness, orientation, behavior.
Temperature:
 Check every 4 hr. or every visit; call primary health care provider if
temperature is >38°C (>101°F), fever is unresponsive to
acetaminophen, or patient shows a decline in hemodynamic status.
Diagnostic Studies
 Monitor complete blood count (CBC
 Call physician if ANC is <1000, significantly different from previous
 count, or whenever patient becomes symptomatic (e.g., febrile).
 Monitor globulin, albumin, total protein levels.
 Monitor all culture and sensitivity reports.
 Monitor radiology reports.
Precautions
 Environment and Staff
 Thorough hand hygiene must be done by everyone before
entering patient’s room each and every time.
 Allow no one with a cold or sore throat to care for the patient or
Allow no one with a cold or sore throat to care for the patient or
to enter room or come in contact with patient at home.
 Care for neutropenic patients before caring for other patients
(as much as possible).
 Use private room for patient if ANC is <1000.
  Allow no fresh flowers (stagnant water).
 Change water in containers every shift (include O2
humidification systems every 24 hr.).
 Ensure room is cleaned daily.
 Dietary
 Provide low microbial diet.
 Eliminate fresh salads and unpeeled fresh fruits or vegetables.
 Patient
 Avoid suppositories, enemas, rectal temperatures.
 Practice deep breathing (with incentive spirometer) every 4 hrs.
 while awake.
 Ambulate; wear high-efficiency particulate air (HEPA) filter
mask if neutropenia is severe. Prevent skin dryness with water-
soluble lubricants, especially in high-risk areas (e.g., lips,
corners of mouth, elbows, feet, bony prominences).
 Hygiene
 Provide meticulous total body hygiene daily (preferably with
antimicrobial solution), including perineal care after every bowel
movement.
 Provide thorough oral hygiene after meals and every 4 hrs.
while awake; warm saline, or salt and soda solution, is effective;
avoid use of lemon-glycerin swabs, commercial mouthwashes,
and hydrogen peroxide.
 Intravenous (IV) Therapy
  Do not use plastic cannulas for peripheral IVs when ANC
is<500 (if possible per agency); a central vascular access
device is preferred for long-term or intensive IV therapy.
 Inspect IV sites every shift; monitor closely for any discomfort;
erythema may not be present.
 Maintain meticulous IV site care.
 Cleanse skin with antimicrobial solution before venipuncture
(unless patient is allergic).
 Moisture-vapor–permeable dressings are permissible with strict
adherence to institutional protocol.
 Change IV tubing per institution policy, using aseptic technique.
 Administer antimicrobial agents on time
4. Leukocytosis
 Leukocytosis means you have a high white blood cell count. This
means you have more white blood cells than normal.
 Leukocytosis is a normal immune response and isn’t always a cause
for concern.
 Most of the time, it means that your body is fighting off infection
or inflammation.
 However, there are times when a high white blood cell count could
indicate something more serious, such as leukemia.
5. leukemia
Definition
 Leukemia (literally, white blood) is a group of malignant disorders of
WBCs. in leukemia, the usual ratio of greater numbers of red blood
 cells than white blood cells is reversed. bone marrow is gradually
replaced by immature, abnormal cells
 Eventually, these abnormal cells spill into the circulation and invade
other organs such as the liver, spleen, and lymph nodes. If the
disease is not treated, leukemia cells replace all normal blood cells,
leading to death.
Causes of leukemia
 The cause of most leukemia is unknown.
 exposure to chemicals such as benzene,
 genetic factors
 immune disorders,
 certain cancer drugs
 exposure to large doses of radiation.
Pathophysiology
 Leukemia begins with the malignant transformation of a single stem
cell.
 leukemic cells proliferate slowly, but do not become functional WBCs.
 Leukemic cells leave the bone marrow and infiltrate other tissues
such as central nervous system, testes, skin, GI tract, and the lymph
nodes, liver, and spleen.
 death usually results from internal hemorrhage and infections.
Types of leukemia
 Leukemia are classified by their onset and duration
 acute
 chronic
 by the types of abnormal cells
  Myelogeno
 lymphocytic

1. Acute lymphocytic leukemia (ALL)

 It is the most common type of leukemia in young children
before age of 14 years.
 It also affects adults. ALL is manifested by fever, pallor,
bleeding, anorexia, and CNS symptoms as lethargy.
 Treated by chemotherapy; stem cell transplant (SCT) or bone
marrow transplant (BMT).
2. Acute myeloid leukemia (AML)
 It occurs in both adults and children. AML is manifested by
fatigue, weakness, headache, and mouth sores.
 Treated by chemotherapy; stem cell transplant (SCT).
3. Chronic lymphocytic leukemia (CLL)
 It is most often over age 55 and it could have no signs or
symptoms and detected during examination.
  It almost never affects children.
 Often requires no treatment; chemotherapy, BMT.
4. Chronic myeloid leukemia (CML)
 It affects mainly adults occurs at age of 25-60 years.
 (Myeloid cell origin in the bone marrow or spinal cord, or a
resemblance to the marrow or spinal cord).
 It could be manifested by fatigue, joint pain, anorexia, fever,
night sweats, and weight loss.
 Treated by interferon- alpha chemotherapy and SCT.
The manifestations of leukemia result from anemia, Infection, and
bleeding
 Anemia causes: pallor, fatigue, tachycardia, malaise, lethargy, and
dyspnea.
 Infection, due to impaired WBC function, causes fever; night sweats;
ulcers of the mouth and pharynx; and respiratory, urinary tract, and
skin infection.
 Septicemia may develop.
 Thrombocytopenia (low platelet count) increases the risk of bleeding,
leading to bruising, and hematomas, as well as overt and hidden
bleeding into organs
 As leukemic cells infiltrate other organs, pain and other symptoms
may occur.
Diagnostic tests
 Bone marrow aspiration:
 showing a proliferation of immature WBCs confirms acute
leukemia.
 Blood counts:
 show thrombocytopenia and neutropenia
 lumbar puncture:
 detects meningeal involvement.
 A computed tomography scan shows the affected organs, and
cerebrospinal fluid analysis detects abnormal WBC invasion of the
central nervous system.
Treatment of leukemia
 Chemotherapy:
 used to destroy leukemic cells and produce remission.
Chemotherapy interferes with the proliferation of cells in the
bone marrow.
 Radiation therapy:
 Radiation therapy may be used to shrink enlarged lymph nodes
and destroy leukemic cells in the central nervous system (CNS)
 radiation damages the cell DNA so that it is unable to reproduce
and multiply. Although normal cells are affected.
 Biologic therapy:
 such as interferon and interleukins. These agents have multiple
effects, including moderate immune function and slowing
abnormal cell proliferation and growth
 Side effect of interferon therapy includes flu like symptoms,
persistent fatigue and lethargy, weight loss, and muscle and
joint pain.
Nursing diagnosis
 Risk for infection related to compromised immunity
 Bleeding due to decreased platelets
 Risk for disturbed body image related to hair loss secondary to
chemotherapy
 Risk for deficient fluid volume related to potential for diarrhea,
bleeding, infection, and increased metabolic rate
 Anxiety and fear related to unfamiliar experiences and unknown
prognosis
Planning and intervention:
 Risk for infection related to compromised immunity
 Implement neutropenic precautions
 place the client in private room
 always wash hands before touching the client and tell client to
wash his hand before and after eating and after using the
bathroom
 encourage the client to shower daily
  place a mask over client’s mouth and nose if leaving the room
 ensure that no raw fruits or vegetables are served
 minimize invasive procedures
 Bleeding due to decreased platelets
 Assess the following areas thoroughly every shift or visit (with
spot checks throughout the shift if patient is hospitalized), and
notify physician if there is new onset of the following and/or
worsening of status:
 Integument: Petechiae (usually located on trunk, thighs),
ecchymoses
 or hematomas, conjunctival hemorrhages, bleeding
 gums, bleeding at puncture sites (venipuncture, lumbar
puncture,
 bone marrow)
 Genitourinary: Vaginal or urethral bleeding
 Cardiovascular: Hypotension, tachycardia, complaints of
dizziness, epistaxis
 Gastrointestinal: Hemoptysis, abdominal distention, rectal
bleeding
 Prevent Complications
 Avoid aspirin and aspirin-containing medications or other
medications known to inhibit platelet function, if possible
 Genitourinary: Vaginal or urethral bleeding
 Do not give intramuscular injections.
 Do not insert indwelling catheters.
 Take no rectal temperatures; do not give suppositories,
enemas.
 Use stool softeners, oral laxatives to prevent constipation..
 Apply pressure to venipuncture sites for 5 min or until bleeding
has stopped
 Risk for disturbed body image related to hair loss secondary to
chemotherapy
 Provide opportunity for client to express feelings about hair loss
and changing body image
 Offer suggestions such as scarves, turbans. head covering may
increase self-esteem and foster more interactions with others
 the nurse may offer support to enhance the patient’s spiritual
well-being and assist the patient to maintain hope
 Anxiety and fear related to unfamiliar experiences and unknown
prognosis
 Providing emotional support, encourage support a. Discusses
concerns and fears from family and friend
 The nurse also needs to assess how much information patients
want to have regarding the illness, its treatment, and potential
complications.
 Management of pain.
 Uses stress management strategies appropriately
 Discharge from the hospital can also provoke anxiety. Although
most patients are extremely eager to go home,
 Risk for deficient fluid volume related to potential for diarrhea,
bleeding, infection, and increased metabolic rate
 Maintaining fluid and electrolyte balance: - measured
accurately, and daily weights should also be monitored.
 The patient should be assessed for signs of dehydration as well
as fluid overload, with particular attention to pulmonary status
and the development of dependent edema.
  Laboratory test results, particularly electrolytes, blood urea
nitrogen, creatinine, and hematocrit, should be monitored and
compared with previous results.
 Replacement of electrolytes, particularly potassium and
magnesium, is commonly required
 Patients receiving amphotericin or certain antibiotics are at
increased risk for electrolyte depletion
Expected patient outcomes may include:
 Shows no evidence of infection
 Experiences no bleeding
 Maintaining fluid and electrolyte balance
 Copes with anxiety and grief
6. Lymphoma
 types of blood cancer, starts in the lymphatic system and affects the
lymph nodes and lymph tissues
The main types of lymphoma include:
 Non-Hodgkin lymphoma (NHL): The most common type of lymphoma
that usually starts in B or T cells
 Hodgkin lymphoma (HL): One of the most treatable types of cancer,
usually starting in the B cells lymph tissues.
Symptoms
 swollen lymph nodes are common. These lymph nodes may be in the
neck, groin, armpit, chest, or stomach.
 Shortness of breath
 Fatigue
 Fever
  Loss of appetite
 Unintentional weight loss
 Night sweats
lymphoma diagnosis
 Medical history
 Physical exam
 Biopsy of lymph nodes
 Blood tests
 Bone marrow biopsy
 Imaging: MRI, CT, or PET scan
lymphoma treatments
 Chemotherapy
 Targeted drug therapy
 Radiation
 Bone marrow transplant
 Immunotherapy
Nursing management for lymphoma
 Observe for neck vein distention, headache, dizziness, facial edema,
dyspnea, and stridor
 Lymphoma patients are at higher risk for vena cava syndrome in
which the superior vena cava is obstructed from enlarged lymph
nodes. This constitutes a life-threatening emergency and MD should
be notified. Emergency radiation treatment may be ordered.
 Assess and manage pain; teach relaxation techniques, administer
analgesics as necessary
 While the enlarged lymph nodes are usually painless, patients may
experience pain with radiation or chemotherapy treatments.
 Management of pain and reduction of stress is essential to patients to
promote healing and conserve energy.
 Nutrition education; monitor daily weight and caloric intake;
encourage patients to eat small frequent meals and increase protein
intake.
 Patients may experience lack of appetite and diminished nutrition.
Increasing caloric intake promotes healing, provides fuel for energy,
and prevents gastric distention. Offer more palatable options
frequently.
 Provide supportive comfort measures following radiation or
chemotherapy treatments.
 Patients often experience extreme fatigue, nausea and vomiting
following treatment. Assist with ADLs, offer ice chips and antiemetics
as appropriate for nausea.
 Assist with positioning and monitor for skin breakdown
 Fatigue and impaired nutrition cause muscle weakness. Assist
patients to positions of comfort for optimal air exchange and monitor
skin for signs of breakdown due to prolonged bed rest.
 Vitals
 Educate the patient about the disease
 Monitor for respiratory distress
 WBCs multiply rapidly, it can reduce the oxygen carrying capacity of
the red blood cells, resulting in hypoxemia.
C. Bleeding Disorders
 Hemostasis is the process of preventing blood loss from intact
 vessels and of stopping bleeding from a severed vessel. The
prevention of blood loss from intact vessels requires adequate
numbers of functional platelets. Platelets nurture the endothelium and
thereby maintain the structural integrity of the vessel wall.
 Two processes are involved in arresting bleeding: primary and
secondary hemostasis.
 In primary hemostasis, the severed blood vessel constricts.
Circulating
 platelets aggregate at the site and adhere to the vessel and to one
another. An unstable hemostatic plug is formed. For the coagulation
process to be correctly activated, circulating inactive coagulation
factors must be converted to active forms.
 This process occurs on the surface of the aggregated platelets at the
site of vessel injury. The end result is the formation of fibrin, which
reinforces the platelet plug and anchors it to the injury site. This
process is termed secondary hemostasis. The process of blood
coagulation is highly complex. It can be activated by the intrinsic or
the extrinsic pathway. Both pathways are needed for maintenance of
normal hemostasis
 Many factors are involved in the reaction cascade that forms fibrin.
When tissue is injured, the extrinsic pathway is activated by the
release from the tissue of a substance called thromboplastin. As the
result of a series of reactions, prothrombin is converted to thrombin,
which in turn catalyzes the conversion of fibrinogen to fibrin.
  Clotting by the intrinsic pathway is activated when the collagen that
lines blood vessels is exposed. Clotting factors are activated
sequentially until, as with the extrinsic pathway, fibrin is ultimately
formed. Although the intrinsic pathway is slower, this sequence is
probably most often responsible for clotting in vivo.
 The failure of normal hemostatic mechanisms can result in bleeding,
which is severe at times. This bleeding is commonly provoked by
trauma, but in certain circumstances it can occur spontaneously.
When the source is platelet or coagulation factor abnormalities, the
site of spontaneous bleeding can be anywhere in the body.
 In a variety of situations, the bone marrow may be stimulated to
increase platelet production (thrombopoiesis). The increased
production may be a reactive response, as in a compensatory
response to significant bleeding, or a more general response to
increase hematopoiesis, as in iron deficiency anemia. Sometimes,
the increase in platelets does not result from increased production but
from a loss in platelet pooling within the spleen.
 The spleen typically holds about one third of the circulating platelets
at any time. If the spleen is lost (e.g., splenectomy), the platelet
reservoir is also lost, and an abnormally high amount of platelets
enter the circulation. In time, the rate of thrombopoiesis slows to
reestablish a more normal platelet level.
Clinical Manifestations of bleeding disorder
 Signs and symptoms of bleeding disorders vary depending on the
type of defect.
  A careful history and physical examination can be very useful in
determining the source of the hemostatic defect.
 Abnormalities of the vascular system give rise to local bleeding,
usually into the skin. Because platelets are primarily responsible for
stopping bleeding from small vessels,
 patients with platelet defects develop petechiae, often in clusters;
these are seen on the skin and mucous membranes but also occur
throughout the body. Bleeding from platelet.
 Bleeding from platelet disorders can be severe. Unless the platelet
disorder is severe, bleeding can often be stopped promptly when
local pressure is applied; it does not typically recur when the pressure
is released
 In contrast, coagulation factor defects do not tend to cause superficial
bleeding, because the primary hemostatic mechanism are still intact.
Instead, bleeding occurs deeper within the body (e.g., subcutaneous,
or intramuscular hematomas, hemorrhage into joint spaces).
Medical Management
 Management varies based on the underlying cause of the bleeding
disorder.
 If bleeding is significant, transfusions of blood products are indicated.
The specific blood product used is determined by the underlying
defect. In specific situations in which fibrinolysis is excessive,
hemostatic agents such as aminocaproic acid (Amicar) can be used
to inhibit this process.
 This agent must be used with caution because excessive inhibition of
fibrinolysis can result in thrombosis
Nursing Management
 Patients who have bleeding disorders or who have the potential
 for development of such disorders as a result of disease or
therapeutic agents must be taught to observe themselves carefully
and frequently for bleeding.
 avoiding activities that increase the risk of bleeding,
 The skin is observed for petechiae and ecchymosis and the nose and
gums for bleeding.
 Hospitalized patients may be monitored for bleeding by testing all
drainage and excreta (feces, urine, emesis, and gastric drainage)
 Outpatients are often given fecal occult blood screening cards to
detect occult blood in stools.
1. Thrombocytopenia:
Definition:
 Thrombocytopenia is a platelet count of less than 100,000 platelets
per milliliter of blood .it is the most common cause of abnormal
bleeding
Causes:
 Decreased platelet production
 Increased destruction of platelets
 Accumulation of platelets in the spleen
Incidence:
 Immune (or idiopathic) thrombocytopenia purpura is the most
common form of thrombocytopenia. It typically affects young adults
(age 20 to 40); women are affected more frequently than men.
Pathophysiology:
 In immune thrombocytopenia purpura (ITP), platelets are destroyed
much more rapidly than normal. It is an autoimmune disorder in which
platelets are destroyed by the body’s immune system. Antibodies are
developed to protein in the platelet cell membrane; when these
antibodies adhere to the platelet, the spleen identifies the platelet as
a foreign cell, and destroys it
 Immune thrombocytopenia may be an acute or a chronic condition.
Acute ITP is seen in children and is often preceded by a viral
infection. Chronic ITP affects adults and has no known precipitating
factors
Manifestation
 The manifestations of ITP are caused by abnormal bleeding,
particularly into the skin and mucous membranes.
 Purpura: hemorrhage into the tissues
 Ecchymoses (bruises), and petechiae (small, flat, purple, or red spots
on the skin or mucous membranes) develop on the anterior chest,
arms, and neck.
 Other bleeding may occur as well: epistaxis (nosebleed),
menorrhagia (prolonged and heavy menstrual periods), hematuria,
below20,000/mm3
 Spontaneous bleeding into the brain can be fatal
 Associated symptoms such as headache, weight loss, and fever also
may occur gastrointestinal hemorrhage When the platelet count is
less than 5000/mm3
Interdisciplinary care
1. Diagnostic tests
 CBC with platelet count is ordered.
 the platelet count is decreased to less than 100,000/ml.
 A bone marrow examination may be ordered to evaluate
platelet production.
 Tests for an abnormal antibodies, called antinuclear antibodies
(ANA), are done to assess for autoimmunity.
2. Medications:
 Corticosteroids such as prednisone are given to suppress the
immune response and the antibodies targeted for the platelets
 Immunosuppressive drugs such as cyclosporine also may be
used.
3. Treatment
 treatment of the underlying disease.
 Platelet transfusions may be needed to restore the platelet
count and prevent bleeding. Platelets are prepared from fresh
whole blood; one unit contains 30 to 60 ml of platelet
concentrate.
 Plasma exchange therapy, also known as plasma pheresis,
may be done to remove circulating autoantibodies. In this
treatment, the client’s plasma is removed and replaced with
fresh frozen plasma
4. Surgery
 A splenectomy (surgical removal of the spleen) may be necessary.
the spleen is the site of platelet destruction and antibody production.
This surgery often leads to remission or even cure of the disorder.
2. Hemophilia
Definition:
 Hemophilia is not a single disease but a group of hereditary clotting
factor deficiencies. Lack of a clotting factor disrupts
 Blood coagulation, leading to persistent and sometimes severe
bleeding
Causes of Hemophilia
 Inherited. Factors
Pathophysiology
 In hemophilia, the first steps of homeostasis, vasoconstriction, and
the platelet plug, occur normally. The third step, blood clotting, is
disrupted by lack of a specific factor required in the clotting cascade.
 Hemophilia A is the most common type of hemophilia. It is caused by
a deficiency in factor VIII. Hemophilia B (also called Christmas
disease) is less common. It is caused by a deficiency in factor IX.
 The clinical manifestations of hemophilia A and B are the same. Both
are transmitted from mother to son as sex – linked recessive
disorders on the X chromosome
Clinical Manifestations of Hemophilia
 Slow, persistent, prolonged bleeding from the following sites:
 Bleeding in the mouth from a cut or bite or from cutting or losing
a tooth.
 Nosebleeds for no obvious reason.
 Heavy bleeding from a minor cut.
 Bleeding from a cut that resumes after stopping for a short time.
  Signs of internal bleeding include:
 Blood in the urine (from bleeding in the kidneys or bladder)
 Blood in the stool (from bleeding in the intestines or stomach).
 Bleeding in the Joints
 Bleeding in the knees, elbows, or other joints is another
common form of internal bleeding in people with hemophilia.
 Swelling continues as bleeding continues in the joint
 Cerebral Bleeding
 The signs and symptoms of bleeding in the brain include:
 Long-lasting painful headaches or neck pain or stiffness
 Repeated vomiting
 Changes in behavior or being very sleepy
 Sudden weakness or clumsiness of the arms or legs or difficulty
walking
 Double vision
 Convulsions or seizures
Diagnostic Studies of Hemophilia
 The platelet count is measured; it frequently is within normal limits.
 Coagulation studies, including the PTT, bleeding time, and
prothrombin time are done as screening tests for hemophilia. They
often demonstrate prolonged bleeding times
 Clotting factors assays are done to identify specific clotting factor
deficiencies
Medical management:
 In the past, the only treatment for hemophilia was infusion of fresh
frozen plasma,
  factor VIII and factor IX concentrates are available to all blood banks
or, cryoprecipitates
 Aminocaproic acid (EACA; Amicar) is fibrinolytic enzyme inhibitor that
can slow the dissolution of blood clots that do form; it is very effective
as an adjunctive measure after oral surgery. It is also useful in
treating mucosal bleeding
Nursing Management
 Most patients with hemophilia are diagnosed as children. They often
require assistance in coping with the condition because it is chronic,
encouraged to be self-sufficient and to maintain independence.
 These patients need extensive teaching about activity restrictions and
self-care measures to diminish the chance of hemorrhage and
complications of bleeding.
 Patients with hemophilia are instructed to avoid any agents that
interfere with platelet aggregation, such as aspirin, NSAIDs, herbs,
nutritional supplement, and alcohol.
 Dental hygiene is very important as a preventive measure because
dental extractions are so hazardous.
 Nasal packing should be avoided because bleeding frequently restart
when the packing is removed.
 All injections should be avoided; invasive procedures (e.g.,
endoscopy, lumbar puncture) should be minimized or performed after
administration of appropriate factor replacement. If the patient has
had recent surgery.
  the nurse frequently and carefully assesses the surgical site for
bleeding. Frequent vital sign monitoring is needed until the nurse is
certain that there is no excessive postoperative bleeding..
 Analgesics are commonly required to alleviate the pain associated
with hematomas and hemorrhage into joints warm baths promote
relaxation, improve mobility, during bleeding episodes, apply of cold
are used
3. VON WILLEBRAND’S DISEASE
 Von Willebrand’ s disease, a common bleeding disorder affecting
males and females equally, the disease is caused by a deficiency of
von Willebrand factor (VWF), which is necessary for factor VIII
activity.
 VWF is also necessary for platelet adhesion at the site of vascular
injury. Although synthesis of factor VIII is normal, its half-life is
shortened; therefore, factor VIII levels commonly are mildly low (15%
to 50% of normal).
Clinical Manifestations
 Patients commonly have nosebleeds
 excessively heavy menses.
 postoperative bleeding, although they do not suffer from massive soft
tissue or joint hemorrhages.
 Bleeding from a dental extraction,
Medical Management
 administration of cryoprecipitate, which contains factor VIII,
fibrinogen, and factor XIII (or fresh frozen plasma, if cryoprecipitate is
unavailable).
  N.B: - Replacement continues for several days to ensure correction of
the factor VIII deficiency; up to 7 to 10 days of treatment may be
necessary after major surgery.
 Desmopressin provides a transient increase in factor VIII coagulant
activity and may also correct the bleeding time. It can be
administered as an intravenous infusion or intranasally.
 With major surgery or invasive procedures, both desmopressin and
cryoprecipitate may be needed to prevent hemorrhage.
4. Acquired Coagulation Disorders
 LIVER DISEASE With the exception of factor VIII, most blood
coagulation factors are synthesized in the liver.
 Therefore, hepatic dysfunction (due to cirrhosis, tumor, or hepatitis)
can result in diminished amounts of the factors needed to maintain
coagulation and hemostasis.
 Prolongation of the PT, unless it is caused by vitamin K deficiency,
may indicate severe hepatic dysfunction.
 Although minor bleeding is common (e.g., ecchymoses), these
patients are also at risk for significant bleeding, related especially to
trauma or surgery.
 Transfusion of fresh frozen plasma may be required to replace
clotting factors and to prevent or stop bleeding.
 Patients may also have life-threatening hemorrhage from peptic
ulcers or esophageal varices. In these cases, replacement with fresh
frozen plasma, PRBCs, and platelets is usually required
VITAMIN K DEFICIENCY
 The synthesis of many coagulation factors depends on vitamin K.
 Vitamin K deficiency is typical in malnourished patients, and some
antibiotics decrease the intestinal flora that produce vitamin K,
depleting vitamin K stores.
 Administration of vitamin K (phytonadione [e.g., Mephyton], either
orally or as a subcutaneous injection) can correct the deficiency
quickly; adequate synthesis of coagulation factors is reflected by
normalization of the PT.
5. Disseminated intravascular coagulation (DIC)
Definition:
 (DIC) is a complex disorder characterized by simultaneous blood
clotting and hemorrhage. It is complication of other disorders, such as
shock, sepsis.
Risk factors for disseminated intravascular coagulation
 Hypovolemic or septic shock
 Infection and sepsis
 Malignancy
 Obstetric complications
 Trauma
 Liver disease
 Hematologic or immune disorders
 Acute respiratory distress syndrome
Pathophysiology
 DIC is a complex process in which the intrinsic and / or extrinsic
clotting cascades are activated. Widespread clotting occurs within
small blood vessels.
 Clotting obstructs small blood vessels in the organs, leading to tissue
ischemia, infarction, and necrosis.
 The widespread clotting activates the fibrinolytic pathway that
normally breaks down existing clots.
 Fibrinolytic products interfere with platelet function.
 Clotting factors are depleted by the abnormal coagulation process,
leading to bleeding.
 Thus, in the client with DIC, both intravascular clotting and
hemorrhage are occurring at the same time
Clinical Manifestation of (DIC)
 Petechiae, purpura, ecchymoses
 Bleeding from wounds
 Tachycardia, hypotension
 Cold, mottled fingers and toes
 Tachypnea
 Obvious or occult blood in vomitus and / or stool
 Abdominal distention
 Hematuria
 Oliguria, renal failure
 Anxiety, confusion
 Decreased level of consciousness
Diagnostic studies:
 Decreased platelet count- less than 100,000/µl
 Reduced fibrinogen levels – less than 150 mg /dl
 Prolonged prothrombin time- more than 15 seconds
 Prolonged partial thromboplastin time- more than 60 to 80 seconds
 Positive D- dimmer test (specific fibrinogen test for DIC)-positive at
less than 1:8 dilution.
Treatment
 Treatment focuses on treating the underlying disease and interrupt in
the clotting / bleeding process.
 If liver function is intact, it can restore depleted clotting factors in 24 to
48 hours.
 Fresh frozen plasma and platelet concentrates are given to control
bleeding.
 Heparin also may be ordered heparin interferes with the clotting
cascade and may prevent depletion of clotting factors due to
uncontrolled clotting. It is used when bleeding is not controlled by
plasma and platelets, and when the client is at risk for tissue necrosis
and gangrene.
Diagnosis
 NURSING DIAGNOSES
Based on the assessment data, major nursing diagnoses for the patient
with DIC may include the following:
 Risk for deficient fluid volume related to bleeding
 Risk for impaired skin integrity related to ischemia or bleeding
  Potential for excess fluid volume related to excessive blood/ factor
component replacement
 Ineffective tissue perfusion related to microthrombi
 Anxiety and fear of the unknown and possible death
Planning and Goals
 Major patient goals include maintenance of hemodynamic status,
maintenance of intact skin maintenance of fluid balance, maintenance
of tissue perfusion, enhanced coping, and absence of complications
1. Nursing Diagnosis: Potential for fluid volume deficit related to
bleeding
 Goals: Hemodynamic status maintained
 Urine output ≥30 mL/hr.
 Nursing Interventions
 Avoid procedures/activities that can increase intracranial
pressure (e.g., coughing, straining to have a bowel movement
 Monitor vital signs closely, including neurologic checks:
 Monitor hemodynamics
 Monitor abdominal girth
 Monitor urine output
 Avoid medications that interfere with platelet function if possible
(e.g., ASA, NSAIDs, beta-lactam antibiotics).
 Avoid rectal probes, rectal medications.
 Avoid IM injections.
 Monitor amount of external bleeding.
  Monitor number of dressings, % of dressing saturated; time to
saturate dressing is more objective than “dressing saturated a
moderate amount.”
 Monitor suction output, all excreta
 Monitor pad counts in menstruating females. Females may receive
progesterone to prevent menses.
2. Nursing Diagnosis: Potential for fluid volume excess
 Goals: Absence of edema; absence of rales; Intake not greater than
output
 Auscultate breath sounds every 2–4 hr. Crackles can develop quickly
 Monitor extent of edema
 Monitor volume of IVs, blood products Helps prevent fluid overload.
 decrease volume of IV medications if possible.
 Administer diuretics as prescribed.
 Decreases fluid volume
3. Nursing Diagnosis: Potential for fear of unknown and possible
death
 Goal: Fears verbalized/identified; maintain realistic hope
 Identify previous coping mechanisms, if possible. Encourage patient
to use them as appropriate.
 Explain all procedures and rationale for these in terms patient and
family can Decreased knowledge and uncertainty can increase
anxiety understand.
 Assist family in supporting patient Family can be useful in assisting
patient to use coping strategies and to maintain hope.
 Use services from behavioral medicine, chaplain as needed.
  Identifying previous stressful situations can aid in recall of successful
coping mechanisms.
4. Nursing Diagnosis: Potential for impaired skin integrity secondary
to ischemia or bleeding
 Goals: Skin integrity remains intact; oral mucosa remains intact
 Assess skin, with particular attention to bony prominences, skin
folds.
 Prompt identification of any area at risk for skin breakdown or
showing early signs of breakdown can facilitate prompt intervention
and thus prevent complications
 Reposition carefully; use pressure-reducing mattress.
 Perform careful skin care every 2 hr., emphasizing dependent areas,
all bone prominences, perineum
 Use lamb’s wool between digits, around ears, as needed
 Use prolonged pressure after injection or procedure when such
measures must be performed (at least 5 min).
 Initial platelet plug is very unstable and easily dislodged, which can
lead to increased bleeding.
 Administer oral hygiene carefully
6. Thrombotic Disorders
 Thrombotic disorders are conditions which interfere with hemostasis,
the natural process of blood clotting.
 When hemostasis functions normally, blood clots, or thrombi, form
when there is damage to a blood vessel, but do not produce clots that
block normal vessels.
  Abnormalities can result in the inability to form clots or in the
excessive formation of clots, both of which can cause serious
damage and may even be fatal.
 Several inherited or acquired deficiency conditions, including hyper
homocystinemia, anti-thrombin III (AT III) deficiency, Protein C
deficiency, activated Protein C (APC) resistance, factor V Leiden, and
Protein S deficiency can predispose a patient to repeated episodes of
thrombosis.
a) Hyper Homocystinemia
 Homocysteine is an amino acid produced when proteins are broken
down. A high homocysteine level, also called hyper homocystinemia,
can contribute to arterial damage and blood clots in your blood
vessels. High homocysteine levels usually indicate a deficiency
in vitamin B-12 or folate.
 A normal level of homocysteine in the blood is less than 15
micromoles per liter (mcmol/L) of blood
 N.B In hyper homocystinemia, the endothelial lining of the vessel
walls is denuded; this can precipitate unnecessary thrombus
formation.
 used dietary supplements with folic acid and vitamin B6 low incidence
of thrombotic condition.
b) Antithrombin III deficiency
 Antithrombin is a protein that inhibits thrombin and certain
coagulation factors. AT III deficiency is an extremely common
hereditary condition that can cause venous thrombosis, particularly
 when the level is less than 60% of normal
  The most common sites for thrombosis are the deep veins of the leg.
c) PROTEIN C DEFICIENCY
 Protein C is an enzyme that, when activated, inhibits coagulation (one
from anticoagulant in the blood). can be inherited or from other
conditions.
 When levels of Protein C are deficient, the risk of thrombosis
increases, and thrombosis can often occur spontaneously.
Activated protein C resistance and factor V Leiden mutation
 Activated protein C (APC) resistance is a common condition APC is
an anticoagulant,
 and resistance to APC increases the risk for venous thrombosis. A
molecular defect in the factor V gene has been identified in most
(90%) of those with APC resistance; this defect is called factor V
Leiden mutation.
 People who are homozygous for the factor V Leiden mutation are at
extremely high risk for thrombosis.
Medical Management
 The primary method of treating thrombotic disorders is
anticoagulation. Anticoagulation therapy is not without risks; the most
significant risk is bleeding.
 Unfractionated Heparin Therapy. Heparin is a naturally occurring
anticoagulant that enhances AT III and inhibits platelet function. To
prevent thrombosis, heparin is typically given as a subcutaneous
injection, two or three times daily. Should monitor with PTT aiming for
1.5 to 3.
Low-Molecular-Weight Heparin Therapy
 SC administration. LMWH is also being increasingly used as “bridge
therapy” when patients receiving anticoagulation therapy (warfarin)
require an invasive procedure (e.g., biopsy, surgery). In this situation,
warfarin is stopped and LMWH is used in its place until the procedure
is completed. After the procedure, warfarin therapy is resumed.
LMWH is discontinued after a therapeutic level of warfarin is
achieved.
Warfarin (Coumadin) Therapy
 are antagonists of vitamin K and therefore interfere with the synthesis
of vitamin K–dependent clotting factors.
 it must be co- administration with another anticoagulant until
international normalized ratio (INR) reaches the desired therapeutic
range, the heparin is stopped. (Typically using an INR of 2.0 to 3.0)
Nursing Management
 Patients with thrombotic disorders should avoid activities that
promote circulatory stasis (e.g., immobility, crossing the legs).
 When patients with thrombotic disorders are hospitalized, frequent
assessments should be performed for signs and symptoms of
beginning thrombus formation, particularly in the legs (DVT), lungs
(pulmonary embolism).
 Ambulation or range-of-motion exercises
 use of elastic compression stockings should be initiated promptly to
decrease stasis.
 Prophylactic anticoagulants are commonly prescribed.
Therapies for Blood Disorders
 SPLENECTOMY
 The surgical removal of the spleen
 treatment for other hematologic disorders. For example, an
enlarged spleen may be the site of excessive destruction of
blood cells. If the destruction is life threatening, surgery may be
lifesaving.
 This is the case in autoimmune hemolytic anemia or ITP when
these disorders do not respond to more conservative
measures, such as corticosteroid therapy. Some patients with
severe anemia due to inherited RBC defects (e.g., thalassemia)
may also benefit from splenectomy.
 THERAPEUTIC APHERESIS
 Apheresis: - meaning separation. In therapeutic
 apheresis (or pheresis), blood is taken from the patient and
passed through a centrifuge, where a specific component is
separated from the blood and removed. The remaining blood is
then returned to the patient Sometime
 plasma is removed rather than blood cells—typically so that
specific, abnormal proteins within the plasma will be transiently
 lowered until a long-term therapy can be initiated
 BLOOD AND BLOOD COMPONENT THERAPY

Volume replacement and

Cells and plasma, oxygen-carrying capacity;
Whole blood hematocrit about usually used only in significant
40% bleeding (> 25% blood volume
lost)

RBCs with little Symptomatic anemia: platelets

Packed red plasma (hematocrit in the unit are not
blood cells about functional; WBCs the unit may
(PRBCs) 75%); some platelets cause reaction and are not
and WBCs remain functional

Plasma; all
Bleeding in patients with
Plasma (FFP) coagulation factors
coagulation factor deficiencies;
Complement

Fibrinogen ≥ 150
mg/bag, AHF (VIII:C) von Willebrand’s disease
Cryoprecipitat
80–110 units/bag, Hypofibrinogenemia
e
von Willebrand Hemophilia A
factor; fibronectin

Intravenous recurrent infections).

gamma IgG antibodies ITP; primary immunodeficiency
globulin states

Antithrombin III AT III (trace amounts ATIII deficiency with or at risk

concentrate (AT of other plasma for thrombosis
III)
 proteins

 Blood transfusion.
 BLOOD DONATION: - To protect both the donor and the
recipients, all prospective donors are examined and interviewed
before they are allowed to donate their blood.
 A history of viral hepatitis at any time in the past, or a
history of close contact with a hepatitis or dialysis patient
within 6 months.
 A history of receiving a blood transfusion or an infusion of
any blood derivative (other than serum albumin) within 6
months
 A history of untreated syphilis or malaria because these
diseases can be transmitted by transfusion even years
later. - person who has been free of symptoms and off
therapy for 3 years after malaria may be a donor.
 A skin infection, because of the possibility of
contaminating the phlebotomy needle, and subsequently
the blood itself.
 Pregnancy within 6 months, because of the nutritional
demands of pregnancy on the mother
 A history of tooth extraction or oral surgery within 72
hours, because such procedures are frequently
associated with transient bacteremia.
 Recent immunizations, because of the risk of transmitting
live organisms
  history of recent tattoo, because of the risk of blood-borne
infections (e.g., hepatitis, HIV).
 any aspirin or aspirin-containing medications within the
past 3 days. Although aspirin use does not render the
donor ineligible, the platelets obtained would be
dysfunctional and therefore not useful. Aspirin does not
affect the RBCs or plasma obtained from the donor
 All donors are expected to meet the following minimal requirements:
 Body weight should exceed 50 kg (110 pounds) for a standard 450-
mL donation.
 Donors weighing less than 50 kg donate proportionately less blood.
 People younger than 17 years of age are disqualified from donation.
 The oral temperature should not exceed 37.5°C (99.6°F).
 The pulse rate should be regular and between 50 and 100 beats per
minute.
 The systolic arterial pressure should be 90 to 180 mm Hg, and the
diastolic pressure should be 50 to 100 mm Hg.
 The hemoglobin level should be at least 12.5 g/dL for women and
13.5 g/dL for men.
Nursing responsibilities