Hematology

Agenda
Overview of Blood & Blood Components
Another Zen review
Common Diagnostic tests
Cell counts and plasma proteins
Common blood disorders
Case Study
Platelets and Their Functions
Shrink damaged blood vessels
Form hemostatic plugs
Accelerate clotting
Receptors for
Clotting factors
Plasma phospholipids
Factor XIII
Where Do Platelets Originate?
Multi-nucleated
Megakaryocyte
Platelets derived
Via pinching-off
Clot Formation
Intrinsic Pathway Extrinsic Pathway
Conversion of Clot Factors
XII XIIa
XI XIa
IX IXa
VIII VIIIa
X Xa
V
Platelet Phospholipid
Prothrombin
Fibrinogen
XIII
THROMBIN
FIBRIN
Tissue Thromboplastin
Ca++
VII VIIa
How are Platelets Activated?
Several ways, but mainly by exposure to:
Thrombin
ADP
Collagen
Cytokines (eg: PAF)
The Thrombolytic Pathway
XII
XIIa
PLASMINOGEN
PLASMIN
Fibrin Polymers
Some Clinical Points
tissue plasminogen activator (t-PA)

Vitamin K (phytonadione) & babies

von Willebrands Disease
Basics on Blood Dyscrasias
Qualitative
Intrinsic cell abnormalities, or
Plasma component destruction
Quantitative
or cell production or destruction
Eg: myeloproliferative disorders
Causes?
Bone Marrow Depression
Decreased RBCs
anemia

Decreased Platelets
thrombocytopenia

Decreased granulocytes
granulocytopenia
Pancytopenia
Quantitative Cellular Disorders
RBCs
Anemia, hemorrhage, hypervolemia
WBCs
Leukemias
Lymphomas
Platelets
thrombocytopenias


Red Blood Cell Disorders
Classification System (based on RBC size):
Macrocytic

Folic acid, B12 deficiency, liver
disease, alcohol, drugs
Normocytic

Aplastic anemia, chronic renal
failure, hemolytic anemia
Microcytic

Iron deficiency, thallasemia
TYPE MCV Common Causes
Red Blood Cell Disorders
Classification System (based on hemoglobin
concentration):

Hyperchromic

Normochromic

Hypochromic

TYPE MCHC
Macrocytic/Normochromic
Pernicious Anemia

Folate Deficiency
Microcytic/Hypochromic
Iron Deficiency Anemia

Sideroblastic Anemia
Normocytic/Normochromic
Aplastic Anemia

Posthemorrhagic Anemia

Hemolytic Anemia
White Blood Cell Disorders
Abnormalities in number are common
Abnormalities in function are rare
Most frequent: changes in neutrophil count
Leukocytosis Neutropenia
White Blood Cell Disorders
Lymphocyte numbers can also vary:
Lymphocytosis Lymphopenia
-Viral infections
- chronic is worrisome
- why?
-Steroid therapy
-HIV
-Cushings Syndrome
When might other WBC
numbers vary?
Common Diagnostic Blood Tests
RBC count: (4-6 million/mm
3)

used to assess erythropoiesis, anemias,
blood loss.
Hematocrit: (m: 40-54%; f:38-46%))
Used to assess blood loss, polycythemia,
anemia
Hemoglobin content: (m: 13-18 g Hb/100
ml; f: 12-16 g Hb/100 ml)
MCV, MCH, MCHC
Common Diagnostic Blood Tests
Band Cells: immature neutrophils
If these are found in significant numbers,
known as a LEFT SHIFT
Clinical significance?
Mature Neutrophil Band Cell
Common Diagnostic Tests
WBC Differential:
Neuts: infection, inflammation, stress
Eosin: allergies
Baso: polycythemias, allergic reactions
Monos: Lymphoproliferative disorders, some
leukemias
Lympho: infection, immunodeficiencies,
lymphoproliferative disordes, leukemia
Clotting Tests
Bleeding time
Duration of bleeding followed by puncture of
skin
Measured in minutes
What is normal range?
Clotting Tests
Prothrombin Time (PT)
Blood added to citrate, centrifuged
Calcium and Tissue thromboplastin added to
plasma (why?)
11 16 seconds
A measure of the Extrinsic and Common
Pathways


INR (International Normalized Ratio)
Prothrombin time varies; therefore INR
developed to standardize values
THE standard for measuring effectiveness of
oral anticoagulants
2.0 3.0
Clotting Tests
Clotting Tests
Partial Thromboplastin Time (PTT)
Tissue thromboplastin is a complex mixture of
phospholipids and proteins
blood is drawn into citrate, centrifuged, then
calcium added (why?)
Normal is 25 32 seconds
Effectiveness of Intrinsic Pathway
Clotting Tests
Thrombin time
Quantity and activity of fibrinogen
Impt. For Dx, acute leukemia, DIC
Fibrinogen Assay
Amount of fibrinogen available
Impt. For transfusion rxns, DIC, DVT
Platelet Disorders
Thrombocytosis
Thrombocytopathy
Thrombocytopenia
- Production
(aplastic anemia)
- Survival (SLE)
-Iron deficiency anemia
-polycythemia
- Liver failure, uremia, NSAIDs
Three Disorders in Detail:
DIC, ITP, and
Thrombocytopenia
Disseminated Intravascular
Coagulation
Hallmarks:
Excessive clotting, followed by dissolution
Presentation:
Cutaneous oozing, petechiae, cyanosis, N/V,
oliguria, bleeding from GI tract
Causes:
Damage to endothelium
Release of tissue thromboplastin
Activation of Factor X

Disseminated Intravascular Coagulation
DIC (Continued)
Tests/Therapies
TESTS:
Platlets?
Prothrombin
Time?
Fibrin
degradation
products?

THERAPIES
Supportive
Fresh-frozen
plasma,
platelets, or
packed red cells
Heparin (Why?)

Idiopathic Thrombocytopenic
Purpura
Hallmarks:
Platelets are destroyed by immune system
Presentation:
Nose/oral bleeding, purpura, petechiae, excessive
menstruation
Causes:
Usually, preceded by a viral infection (rubella) or live
viral vaccine (ACUTE)
Immune disorders, such as SLE, HIV (CHRONIC)

ATP for ITP:
A antibodies (IgG) develop to platelets

T Trapped platelets in spleen, liver

P Phagocytosis decreases # of platelets
Tests/Therapies
TESTS:
Platelets
Bleeding time?
IgG level?
Therapies:
Temporization
Immune globulin
Glucocorticoids
Splenectomy
Immunosupressants
Thrombocytopenia
Hallmarks:
Deficient number of platelets
Presentation:
Sudden onset bleeding or blood blisters
Bleeding into any mucous membrane
Causes:
Congenital neonatal rubella, maternal thiazides
Acquired aplastic anemia, nutritional
deficiency, viral infection
Tests/Therapies
Tests:
Platelet count
Bleeding time?
Prothrombin, partial
thromboplastin?
Platelet survival
studies
Therapies:
Corticosteroids
(increase platelet
production
Lithium or folate
IV gamma globulin
Anemias
Iron deficiency
Usually, due to loss of iron thru GI/GU bleeds

Pernicious Anemia
Abnormal erythrocyte maturation
Autoimmune in origin, results in loss of B12
Aplastic
Decreased RBC precursors in marrow
Case Study 1
Case history:
13 yr old male, undue bleeding, nosebleeds
Painful swelling of joints, joint tenderness
Hematuria, ecchymoses, inability to flex/extend
elbows
Case Study 1
Questions:
What tests would you order and why?

How would you differentiate the general
cause of the excessive bleeding?

What therapies might you order?
Case Study 2
A 47 yr old male presents with gradual
onset dyspnea on exertion and fatigue
Also complains of frequent dyspepsia with
occasional epigastric pain
Lab results:
WBC/platelets normal; HCT 29%; MCHC
slightly decreased, RDW markedly increased,
reticulocyte <2%
Smear appears normal
Case Study 2
What might the hematologic findings
indicate?

What should be done to further evaluate?

How should patient be managed?