Hematology: Mohamad H Qari, MD, FRCPA

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Hematology

Mohamad H Qari, MD, FRCPA


Hematology
Cellular Components
Components of Blood
Red Blood Cells
White Blood Cells
Inflammatory process
Coagulation

Blood Typing
Clotting disorders
Hematology
Spleen

Blood Kidneys
Hematopoietic
System

Bone
Liver
Marrow
Hematopoeisis
Three developmental periods
Mesoblastic
Blood islands of yolk sac
Primarily RBC production
Embryonic hemoglobin produced
Hepatic
At 6 weeks cell production in liver
Fetal hemoglobin produced
Spleen, thymus, lymph nodes also active prod.
Myeloid
At 5th month Bone Marrow becomes site of prod.
Liver & spleen now Extramedullary
Hemoglobin A (22)
Requirements For
Hematopoiesis
Stem Cells
Growth Factors
Microenviroment
Stem Cell theory
Pluripotent stem cell originator of all cells
Gives rise to cells committed to specific
development
Research supporting the theory
PHSC has self-maintaining ability
Committed cells differentiate continuously/ No
Reverse Flow
Stem cells in Research
Pluripotent cells derived from:
Cell mass of embryos at blastocyst stage (IVF)
Fetal tissue from terminated pregnancy
Application
Identification of factors of cellular decision-making
Development and testing of drugs
Generation of cells and tissue for therapy
Cytokines & Growth Factors
Cytokines stimulate or inhibit
Prevent apoptosis
Colony-Stimulating Factors produced by
many different cells
Interleukins numbered according to discovery
Components of Blood
Plasma Components
Plasma
Transport mechanism Water
90%
90-92% water.
6-7% proteins
2-3%
Fats
Carbohydrates
(glucose)
Electrolytes
Gases (O2, CO2)
Chemical messengers

Other Protein
3% 7%
Cellular Components
Pluripotent Stem Cell

Myeloid Multipotent Common Lymphoid


Stem Cells Stem Cells

Unipotent Lymphocytes
Progenitors

WBCs
RBCs
Basophils
Eosinophils
Thrombocytes Erythrocytes
Neutrophils
Monocytes Platelets Erythropoietin
Components of Blood
Red Blood Cells
Erythrocyte
Hemoglobin O2
bearing molecule
Comprised of 4
subunits:
Globin (binds to
1 O2 molecule)
Heme (iron)
100% saturation = 4
globin subunits
carrying O2
Each gram of Cellsalive.com
hemoglobin =
1.34 ml O2
Blood Products and Blood Typing

Blood Types
Antigens
A, B, AB, O
Rh factor
Rh+ = ~85%
Rh- = ~15%

Blood transfusion
Components of Blood
Red Blood Cells
(cont.)
Oxygen Transport
Oxy-hemoglobin
dissociation curve
2,3-DPG
Components of Blood
White Blood Cells (Leukocytes)
Margination
Phagocytosis

The macrophage is using its internal


cytoskeleton to envelop cells of the fungus
Candida albicans.
Components of Blood
Leukocytes (cont.)
White Blood Cell Count
Leukopoiesis
Granulocytes
Neutrophil
Basophil
Eosinophil
Monocytes
Human Neutrophil:
Lymphocytes Phagocytosis of Strep pyogenes
Components of Blood
Leukocytes (cont.)
Immunity
Subpopulation of lymphocytes known as T cells and B cells
T cells develop cellular immunity.
B cells produce humoral immunity
Components of Blood
Inflammatory Process
MAST CELLS.
Immunoglobulin E antibody
IgE,
Antigens
Antigens stick to the mast cell IgE
antibodies, causing granules in the
mast cell to fire their contents into
the surrounding tissue.
This releases a host of inflammatory
materials - leukotrienes, tumor
necrosis factor, interleukin-4 and
other cytokines that turn on other
inflammatory cells.
These materials cause fluid to leak
from the capillaries and white cells
including neutrophils, T cells and
eosinophils to leave the circulation.
The end result is a "local
inflammatory response", a red, itchy
welt.

Cellsalive.com
Components of Blood
Hemostasis-
3 mechanisms
Vascular spasm
Contraction of
tunica media
Platelet plug
Platelet
aggregation
Coagulation
Formation of fibrin
clot
Components of Blood
Hemostasis (cont.)
Fibrinolysis
Lysis of clot (plasmin)
Thrombosis
Thrombolytics
Medications affecting clot formation
Alter the enzyme
on the platelet.
Affect the coagulation cascade.
Enhance clotting.
Coagulation Cascade -
Synopsis
This scanning electron micrograph shows the fine structure of
a blood clot. Platelets released from the circulation and exposed
to the air use fibrinogen from the blood plasma to spin a mesh
of fibrin.
General Assessment -
Management
Focused History and
Physical Exam
SAMPLE history
Hematological
disorders are rarely
the chief complaint.
Physical exam
Evaluate nervous
system function.
Skin signs
General Assessment - Management
Physical Exam (cont.)
Lymphatic signs
Gastrointestinal signs
Epistaxis
Atraumatic bleeding gums
Thrush
Atraumatic splenic/hepatic pain
Musculoskeletal signs
Arthralgia (rheumatoid arthritis)
Hemarthrosis
Cardiorespiratory signs
Tachycardia, cx pn, dyspnea - CHF
Genitourinary signs
Hematuria
Menorrhagia
Infection
Blood Products and Blood Typing

Blood Typing - ABO


Blood type Antigen present Antibody
on erythrocyte present in
serum
O None Anti-A, Anti-B
AB A and B None
B B Anti-A
A A Anti-B
Blood Products and Blood Typing
Compatibility Among ABO Blood Groups
Reaction with serum of Recipient
Donor Cells AB B A O
AB - + + +
B - - + +
A - + - +
O - - - -
-= no reaction
+ = reaction
Blood Products and Blood Typing

Brady; Paramedic Care Principles and Practice


Diseases of Erythrocytes
Anemias
Anemia is a sign, not a separate disease process.
Signs and symptoms may not be present until the body is
stressed.
Differentiate chronic anemia from acute episode.
Treat signs and symptoms.
Maximize oxygenation and limit blood loss.
Establish IV therapy if indicated.
Diseases of Erythrocytes

Brady; Paramedic Care Principles and Practice


Diseases of Erythrocytes
Sickle Cell Disease
Normal red cells maintain
their shape as they pass
through the capillaries
and release oxygen to
the peripheral tissues
(upper panel).
Hemoglobin polymers
form in the sickle rell
cells with oxygen
release, causing them to
deform. The deformed
cells block the flow of
cells and interrupt the
delivery of oxygen to the
tissues (lower panel).
Diseases of Erythrocytes
Sickle Cell Disease (cont.)
Sickle cell crises
Vaso-occlusive
Musculoskeletal/abdominal pain
Priapism
Renal/cerebral infarctions
Hematological
Lowered hemoglobin
Splenic sequestration
Infectious
Management
Follow general treatment guidelines prn.
Consider analgesics.
Diseases of Erythrocytes
Polycythemia
Overproduction of erythrocytes.
Occurs in patients > 50 years old or with
secondary dehydration.
Most deaths due to thrombosis

Results in bleeding abnormalities:


Epistaxis, spontaneous bruising, GI bleeding.
Management:
Follow general treatment guidelines.
Diseases of Leukocytes
Leukopenia/Neutropenia
Too few white blood cells or neutrophils.
Follow general treatment guidelines and
provide supportive care.
Leukocytosis
An increase in the number of circulating
white blood cells, often due to infection.
Leukemoid reaction
Diseases of Leukocytes
Leukemia
Cancer of hematopoietic cells
Initial presentation
Acutely ill, fatigued, febrile and weak, anemic.
Thrombocytopenia

Often have a secondary infection.

Management
Follow general treatment guidelines.
Utilize isolation techniques to limit risk of infection.
Diseases of Leukocytes
Lymphomas
Cancers of the lymphatic system
Hodgkin's
Non-Hodgkins
Presentation
Swelling of the lymph nodes
Fever, night sweats, anorexia, weight loss, fatigue, and
pruritis
Management
Follow general treatment guidelines.
Utilize isolation techniques to limit risk of infection.
Clotting Disorders
Thrombocytosis and Thrombocytopenia
Thrombocytosis
An abnormal increase in the number of platelets
Thrombocytopenia
An abnormal decrease in the number of platelets
Sequestration
Destruction (ITP)
Decreased production
Management
Provide supportive care and follow general treatment
guidelines.
Clotting Disorders
Hemophilia
Deficiency or absence of a blood clotting factor
Deficiency of factor VIII causes hemophilia A.
Deficiency of factor IX causes hemophilia B.
Deficiency is a sex-linked, inherited disorder.
Defective gene is carried on the X chromosome.
Signs & Symptoms
Numerous bruises, deep muscle bleeding, and joint
bleeding.
Clotting Disorders
Hemophilia (cont.)
Management
Treat the patient similarly to others.
Administer supplemental oxygen.
Establish IV access.
Be alert for recurrent or prolonged bleeding, and prevent
additional trauma.
Von Willebrands Disease
Deficient component of factor VIII
Generally results in excessive bleeding.
Generally is not serious; provide supportive care.
Clotting Disorders
Disseminated Intravascular Coagulation
Components of Blood
Leukocytes (cont.)
Autoimmune disease
May be specific or general
Alterations in the immune process
Immunosuppression
HIV
Anti-rejection medication
Chemotherapy/Cancer

System activation of coagulation cascade.


Anticoagulants/Antiplatelets
Classifying agents
Anticoagulants
Block synthesis and activation of clotting
factors
Antiplatelets
Interfere with platelet aggregation or activation
Fibrinolytics
Dissolve fibrin component of thrombi
Anticoagulants/Antiplatelets
Anticoagulants
Warfarin
Blocks activation of Vit K
Interferes w/ coagulation factors II, VII, IX, and X
Unfractionated Heparin
Binds antithrombin III (AT-III)
Inhibits coagulation factors IX and X and thrombin
Hirudin (medicinal leech)
Prevents thrombin binding
Anticoagulants/Antiplatelets
Antiplatelets
Aspirin
Prevents platelet activation through
cyclooxygenase blockade.
Prevents production of thromboxane A2
Allows prostacyclin synthesis
Ticlodipine
Deforms platelet membrane fibrinogen receptor
Anticoagulants/Antiplatelets
Antiplatelets (cont.)
Glycoprotein IIb-IIIa Receptor Inhibitors
G IIb-IIIa = platelet membrane receptor of fibrinogen
Abciximab
Eptifibatide
Tirofiban

Fibrinolytics
Activate plasminogen to plasmin = fibrinolysis
Streptokinase
Anistreplase
Tissue Plasminogen Activator
Reteplase
Urokinase
Hemorrhage
Class I Class II Class III Class IV
Blood Loss (ml) Up to 750 750-1500 1500-2000 >2000
Blood Loss (% Up to 15% 15%-30% 30%-40% >40%
volume)
Pulse <100 >100 >120 >140
BP Normal Normal Decreased Decreased
Pulse Pressure Normal or Decreased Decreased Decreased
increased
Resp. Rate 14-20 20-30 30-40 >35
Urine Output (ml/hr) >30 20-30 5-15 Negligible
Mental Status Slightly Mildly Anxious, Confused,
anxious anxious confused lethargic
Fluid Replacement Crystalloid Crystalloid Crystalloid and Crystalloid and
(3:1) rule blood blood
Transfusion Reactions
Hemolytic Reaction
Signs & Symptoms
Facial flushing, hyperventilation, tachycardia, hives,
chest pain, wheezing, fever, chills, and cyanosis
Treatment
Stop transfusion, change all IV tubing, and initiate IV
therapy with normal saline or lactated Ringers.
Consider furosemide, dopamine, and diphenhydramine.
Transfusion Reactions
Febrile Non-hemolytic Reactions
Signs & Symptoms
Headache, fever, and chills
Treatment
Stop transfusion, change all IV tubing, and
initiate IV therapy with normal saline or lactated
Ringers.
Consider Diphenhydramine and an antipyretic.

Observe closely to ensure reaction is non-


hemolytic.

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