CARING FOR CLIENTS WITH

HEMATOLOGIC DISORDERS
RBC DISORDERS
Reduced production of RBCs results in anemia,
of WBCs results in infections, and
of platelets results in bleeding.
• RBC disorders include anemias and poly cythemia vera.
• Anemia is a common hematopoietic disorder in which the
client has a decreased number of RBCs and a low hemo
globin level.

• The causes for anemia are

• a decreased production of RBCs,
• an increased destruction of RBCs, or
• a loss of blood.
Anemias
iron deficiency anemia,
hypoplastic (aplastic) anemia,
pernicious anemia,
acquired hemolytic anemia,
sickle cell anemia.
 IRON DEFICIENCY ANEMIA
• Iron deficiency anemia is the most common type of
anemia and occurs when the body does not have enough
iron to synthesize functional Hgb.
• The decrease in iron may be caused by dietary deficiency.
The most common cause is
blood loss such as in women with heavy menstrual
periods or slow,
chronic blood loss from a peptic ulcer, kidney or
bladder tumor, colon polyp, or colorectal cancer.
• Decreased iron absorption, menstruating women, or an
increased need for iron such as during growth periods or
pregnancy are also causes.
• Iron deficiency anemia is more frequently found in
premature or low-birth-weight infants, adolescent girls,
alcoholic clients, and the elderly.
The symptoms are
fatigue,
palpitations,
tachycardia,
exertional dyspnea,
weakness,
pallor.
Clients with chronic anemia have pica, stomatitis, glossitis,
and brittle hair.

CRITICAL THINKING
How are the symptoms of iron deficiency anemia related to a
decreased red blood cell count and decreased hemoglobin?
Medical management
• An oral iron preparation, usually ferrous sulfate is
ordered.
• These preparations are not given with food or milk
because they interfere with iron absorption.
• The administration of iron with orange juice or
vitamin C–rich drinks increases iron absorption.
Diet
 A diet high in iron is encouraged.
An increase of vitamin C in the diet assists in the
absorption of iron.
Iron pills should not be taken within 2 hours of antacids or
tetracycline or at the same time as foods high in caffeine
(coffee, tea, and chocolate).
If the client has a loss of appetite, small frequent snacks
are tolerated better than three large meals.
Activity: Space daily activities to provide rest periods
between times of exercise.
Iron-rich Foods
 Red meats
 Fish
 Liver
 Raisins
 Apricots
 Dried fruits
 Dark green vegetables
 Dried beans, lentils, chick peas
 Egg yolks
 Iron-enriched whole-grain breads and cereals
APLASTIC ANEMIA
• The bone marrow decreases or stops functioning in a client
with aplastic anemia.
• The client with aplastic anemia has pancytopenia, a decrease
in the number of RBCs, WBCs, and platelets.
• In most cases the cause is unknown, but genetic factors
are suspected.
• Secondary aplastic anemia is caused by exposure to viruses,
chemicals (benzene or airplane glue), radiation, or
medications.
• Some medications that cause aplastic anemia are
chloramphenicol (Chloromycetin), mephenytoin
(Mesantoin), trimethadione (Tridione), mechlorethamine or
nitrogen mustard (Mustargen), methotrexate (Folex PFS), 6-
mercaptopurine or 6-MP (Purinethol), and phenylbutazone
(Butazolidin).
Symptoms in APLASTIC ANAEMIA
fatigue,
weakness,
 palpita tions,
 headaches,
fever,
mouth ulcers,
 petechiae
gingival bleeding, and epistaxis.
These clients are extremely ill.
Diagnosis is confirmed by a bone marrow aspiration
Medical Management
• The cause of aplastic anemia is removed if possible.
• Immunosuppressive therapy with antithymocyte
globulin or ATG (Atgam) and cyclosporine is given to
suppress the reaction causing the aplastic anemia and to
allow the client’s bone marrow to recover.
• A client who has a good response will improve in 3 to
6 months.
• Transfusions of packed red cells and platelets are given
as needed.
 SURGICAL MANAGEMENT
• A bone marrow transplant is performed if the client’s
bone marrow fails to respond to treatment.
• Cyclosporine (Sandim mune), an immunosuppressant, is
given for a bone marrow transplant to decrease the graft
rejection.
• The best response occurs in a young client who has not
previously had a transfusion because transfusions increase
bone marrow graft rejection.
• Bone marrow transplants from a human leukocyte
antigen- (HLA-)matched sibling donor are the treatment
of choice for clients younger than 30 years of age.

• The treatment of choice for an older adult or a client who

does not have an HLA-matched sibling donor is
immunosuppression with ATG and cyclosporine.
• Infections are treated with antibiotics.
• Steroids and androgens are sometimes used to stimulate
the bone marrow.
PERNICIOUS ANEMIA
• The parietal cells of the gastric mucosa secrete a protein
intrinsic factor that is essential for the proper absorption of
vitamin B12.
• Pernicious anemia is an autoimmune disease in which the
parietal cells are destroyed and the gastric mucosa
atrophies. Without the secretion of the intrinsic factor,
vitamin B12 cannot be absorbed in the distal portion of
the ileum.
• The onset of the disease occurs around the age of 60.
• Pernicious anemia occurs most frequently in women of Northern
European descent and some African Americans.

• Pernicious anemia occurs in clients who have had a

gastrectomy with the section of the stomach removed that
secretes the intrinsic factor.
• Pernicious anemia has an insidious onset because the body can
store 3 to 5 years’ worth of vitamin B12 in the liver.
• Neurological changes, paresthesia, and numbness can occur
before the vitamin B12 deficiency becomes anemia.
Symptoms in PERNICIOUS ANAEMIA
• extreme weakness,
• a sore tongue, edema of the legs,
• ataxia,
• dizziness,
• dyspnea,
• headache,
• fever,
• blurred
• vision,
• tinnitus,
• jaundice with pallor,
• poor memory,
• irritability,
• loss of bladder and bowel control.
• The client has decreased sensitivity to heat and pain
because of neurological involvement.

• Clients with pernicious anemia are highly susceptible to

gastric carcinoma and are monitored closely for
symptoms
Medical-Surgical Management
Topical anesthetics are given to relieve oral discomfort
during the acute phase of the disease.
Vitamin B12, cyanocobalamin crystalline (Rubesol-
1000) is given IM until the Hct returns to normal.
• Then it is given monthly for the rest of the client’s
life.
• The frequency of administration depends on the
client’s symptoms and response to the medication.
• Oral administration of vitamin B12 is not effective because
vitamin B12 cannot be absorbed without the intrinsic
factor.
• Folic acid or folate (Folvite) is prescribed.
• Encourage the client to increase folic acid in the diet by
eating green leafy vegetables, meat, fish, legumes, and
whole grains.
• Iron is usually not prescribed because once the condition is
corrected with regular administration of cyanocobalamin,
erythrocytes are produced and the Hgb and Hct return to
normal.
ACQUIRED HEMOLYTIC ANEMIA
• In hemolytic anemias, hemolysis, or destruction of RBCs,
occurs, and iron and hemoglobin are released.
Several causes for acquired hemolytic anemia are
• an autoimmune reaction,
• radiation,
• blood transfusion,
• chemicals, arsenic, lead, or medications.
• Penicillin, and methyldopa (Aldomet) are medications
that cause hemolysis.
• Diagnostic tests reveal a low Hgb and Hct and an
increased level of lactate dehydrogenase (LDH).

• LDH is an enzyme in the heart, liver, kidneys, skeletal

muscle, brain, RBCs, and lungs. As these tissues are
damaged, LDH is released into the bloodstream, causing an
elevated LDH
Medical Management
• Treatment is aimed at removing the cause, if possible.
• Clients are given blood transfusions or the
erythrocytapheresis (a procedure that removes
abnormal RBCs and replaces them with healthy
RBCs) is performed.

Surgical management
• The spleen destroys RBCs. In severe cases of hemolytic
anemia, a splenectomy is performed in an attempt to
stop the destruction of RBCs.
Pharmacological management
• Corticosteroids are administered to decrease the
autoimmune response.
• Folic acid is given to increase the production of RBCs.
SICKLE CELL ANEMIA (INHERITED HEMOLYTIC
ANEMIA)
• Sickle cell anemia is also known as inherited hemolytic
anemia or sickle cell disease.
• This genetic disorder has abnormal hemoglobin S rather
than hemoglobin A in the RBCs.
• Sickle cell anemia is caused by a recessive gene or genes
that are passed through the generations’
• The client with one S gene has sickle cell trait (Hb
sA) and is asymptomatic but is a carrier of the
disease.
• The client with sickle cell anemia has two S genes
(Hb ss) and manifests symptoms.
• Sickle cell tests are done on infants to diagnose sickle cell
trait or disease.

• A screening test to detect the presence of Hb S is the

Sickledex or sickle cell test.

• If Hb S is present, a hemoglobin electrophoresis is done to

distinguish between sickle cell trait and sickle cell disease.
• If the hemoglobin electrophoresis test is negative, the
client has the sickle cell trait and not sickle cell disease.
Situations that precipitate sickle cell crisis are
• dehydration,
• deoxygenation, and
• infections.
• In these situations crystallization of hemoglobin is
promoted, which forces the RBCs to sickle, that is,
become crescent shaped and elongated and obstruct
vessels, especially capillaries.
• The area normally supplied by these obstructed blood
vessels becomes infarcted and ischemic.

• The destruction of sickled RBCs in 12 to 15 days causes

chronic anemia; the heart enlarges in an attempt to
circulate more blood for adequate oxygenation of body
tissues.
Other symptoms include
• fatigue,
• jaundice,
• chronic leg ulcers,
• tachypnea,
• dyspnea, and
• arrhythmias.
When the client experiences a sickle cell crisis, there is
fever,
severe pain, and
loss of blood supply to various organs because of
obstructed vessels.
• Areas most frequently affected are the joints, bone, brain,
lungs, liver, kidneys, and penis.
• Joints become painful, swollen, and immobile.

• Clients experience
cerebrovascular accidents,
renal failure,
pulmonary infarction,
shock, and
priapism (a continuous, painful erection).
• Assess extremity circulation frequently by checking
capillary refill, peripheral pulses, and temperature.
• Application of warm compresses to painful areas relieves
pain.
• Encourage the client to avoid restrictive clothing and
anything that may restrict circulation.
• Teach clients to avoid high altitudes and have adequate
fluid intake.
CLIENT TEACHING IN SICKLE CELL ANAEMIA
Encourage client to avoid high altitudes and non pressurized
airplanes.
Encourage adequate fluid intake because dehydration causes a sickle cell
crisis.
Treat all infections promptly.
Encourage client to avoid tight-fitting, restrictive clothing, strenuous
exercise, smoking, and cold temperatures.
Encourage client to receive yearly flu vaccine and
pneumococcal vaccine.
Encourage client not to smoke.
Teach smoking cessation options.
Medical-Surgical Management
• Infections are treated promptly with antibiotics.
• Large amounts of oral and intravenous fluids (3 to 5 L/day) are
given to remove the by-products of broken RBCs.
• Oxygen is administered based on pulse oximetry and ABGs to
combat deoxygenation.
• Skin grafting is necessary for chronic leg ulcers.
• Genetic counseling is recommended for clients with sickle cell trait
and sickle cell anemia. There may be more openness to counseling
if the counselor is from the same com munity as the client.
• Pharmacological Hydroxyurea reduces the frequency of
painful crisis and the need for blood transfusions in adults.
• Folic acid or folate (Folvite) is administered daily to assist in
the production of RBCs.
• Pentoxifylline (Trental) reduces blood viscosity and increases
RBC flexibility.
• Blood transfusions are given during a crisis.
• Patient-controlled analgesia (PCA) with morphine is
effective during a crisis. The client is progressed from
narcotics to nonnarcotic analgesics as indicated.
Nursing Management
Assess weight, vital signs, apical and peripheral pulses,
breath sounds, color, abdominal tenderness, and signs of
bruising or jaundice.
Review client’s history for possible familial illnesses.
Encourage client to follow treatment regimen.
Monitor laboratory test results.
Administer blood products as ordered and monitor for a
possible reaction.
Keep immunization records current and administer vaccines
as needed, including Haemophilus influenzae, pneumococcal,
meningococcal, hepatitis B, and influenza.
POLYCYTHEMIA
• Polycythemia is a disease in which there is an increased
production of red blood cells. Usually the numbers of
WBCs and platelets are also increased.
• The increase in RBCs increases the blood volume and
viscosity and decreases the ability of the blood to circulate
freely.

There are two types of polycythemia:

1.polycythemia vera (PV) (primary polycythemia)
2.secondary polycythemia.
• The average age for a diagnosis of polycythemia vera is
between the ages of 60 and 65.

• Clients with PV have a mutation of the JAK2 (Janus kinase

2) gene, but the exact role of the mutated gene in the
cause is not known.

• A DNA abnormality occurs in an early marrow cell that

produces all of the blood cells in the individual.
• Secondary polycythemia is a compensatory
mechanism as the body makes more red blood cells in
response to low oxygenation caused by
 long-term hypoxia, as in chronic
obstructive pulmonary disease,
chronic heart failure, smoking, or
living in a high altitude.
• Symptoms of the two types of polycythemia are the same.
• As the blood viscosity and volume increase, the client experiences
headaches,
dizziness,
tinnitus,
blurred vision,
fatigue,
weakness,
pruritus,
exertional dyspnea,
angina, and increased blood pressure and pulse.
• The client’s complexion becomes ruddy (reddish), and
the palms, earlobes, and cheeks are flushed.

• Some clients experience a burning sensation in the feet.

• The client is susceptible to thrombi formation because

of the increased viscosity of the blood and increase in
platelets.
• Even though more RBCs are produced in polycythemia,
the RBCs have a shorter life span than normal.

• When RBCs die, uric acid is released, causing

hyperuricemia (increased uric acid blood level).

• The elevated uric acid levels cause or aggravate gout

symptoms. The Hgb and Hct increase in the same
proportion as the RBCs.
Medical-Surgical Management
• The treatment for polycythemia is phlebotomy, the
removal of blood from a vein.
• Generally 350 to 500 mL of blood is withdrawn at
regular intervals to decrease RBCs.
• A possible side effect of phlebotomy is an increased platelet
count.
Polycythemia complications include
cerebrovascular accident,
thrombosis,
myocardial infarction, and
hemorrhage.

• Clients with PV are more prone to develop leukemia because

of the disease process and medication side effects
CLIENT TEACHINGIN POLYCYTHEMIA
Drink at least 3 L of water daily.
Elevate feet when resting.
Avoid tight or restrictiveclothing.
Wear support hose.
Take medications as ordered.
Report chest pain, joint pain, fever, or activity intolerance
to physician.
Keep appointmentsfor laboratory testing and physician
checks.
Pharmacological
• Low-dose aspirin is given to prevent clot formation.
• Hydroxyurea (Hydrea), a myelosuppressive agent,
reduces the hemoglobin, hematocrit, and platelet count.
• Allopurinol (Zyloprim) is given to decrease the
production of uric acid.
• Pruritus is relieved with the ad ministration of
antihistamines.
Diet
• The client is placed on a diet that has increased calories and
protein.
• A diet low in sodium decreases fluid volume.
• Iron containing foods are avoided.

Activity
• Activities of daily living are adjusted so the client can have
regular periods of rest to relieve fatigue.