Quantitative & Qualitative Disorders of Platelets

Module I
Quantitative & Qualitative

Disorders of Platelets
Quantitative Disorders of Platelets
• Normal platelet count is 150,000 to 450,000
• Thrombocytosis: platelet count greater than 450,000
• Thrombocytopenia: platelet count less than 100,000
• Platelet counts less than 60,000 may bleed unexpectedly during surgery
• Platelet count less than 10,000
• Gingival bleeding
• Epistaxis: nose bleeding
• Ecchymoses: excessive bruising
• Bleeding in C N S
• Hemorrhage
Thrombocytopenia Related to Sample Integrity and Preanalytic
Variables
• Platelet satellitism in peripheral smear
• Re-collect C B C in 3.2% sodium citrate tube and multiply platelet count by 1.11 to correct for
additive volume
- Must be properly mixed
Thrombocytopenia Related to Decreased Production
• Infections with viral

• B M function
• Lack of platelet in
agents
• CMV
B M aplasia
• Lack of megakaryocytes • EBV
• Patients with leukemia • HCV
• HIV
• Megaloblastic conditions
• Varicella
• Cytotoxic agents or • Rubella
chemotherapy
• Bacterial infections
Thrombocytopenia Related to Altered Distribution of Platelets
• Spleen holds one-third of platelet volume

• Enlarged spleen may draw more platelets
• Myeloproliferative disorders
• Extramedullary hematopoiesis
• Hemolytic anemias
• Splenectomy may cause thrombotic problems due to more
platelets in circulation
• Massive transfusion causes coagulation factors to become
diluted
Thrombocytopenia Related to Consumption of Platelets
• Idiopathic thrombocytopenic purpura (I T P) Acute

• Lab: platelet count as low as 20 multiplied by 10⁹/liter
• Clinical: bruising, petechiae, nosebleed
• Age: usually 2 to 6 years
• Often caused by virus
• Lasts 2 to 6 weeks
• I V I G, WinRho, anti-D immune globulin,
splenectomy, or platelet transfusion
• Thrombotic thrombocytopenic purpura (T T P)

• Can occur in platelets with S L E, previous viral infections, or gastric carcinomas
• Platelet count less than 20 multiplied by 10⁹/liter
• P T and P T T within reference range
• T T P clinical findings
• Schistocytes and severe anemia
• Increased L D H
• Decreased haptoglobin
• Hemoglobinuria
• Renal dysfunction may occur
• T T P treatment
• Corticosteroids and plasma
exchange (cryo-poor)
• TTP vs DIC
• Hemolytic uremic syndrome (H U S)

• Usually affects age 6 months to 4 years
• Mimics T T P (no neurologic symptoms) but more
severe renal damage
• Renal damage caused by toxin produced by
E. coli O157:H7 or Shigella
• Patients may present with
• Bloody diarrhea
• Vomiting
• Thrombocytopenia
• Renal failure
• Hemolytic uremic syndrome (H U S)
• Patients may present with (continued)
• Fever
• Abdominal pain
• M H A with schistocytes
• Toxin leads to cell death, especially in kidneys
• Treatment: anti-C5 antibodies, anti-Shiga toxin
Thrombocytosis
• Platelet count is greater than 450 multiplied by 10⁹/liter
• Primary
• Myeloproliferative disorders
• Secondary
• Acute and chronic blood loss
• Chronic inflammatory disease
• Postsplenectomy
• Iron deficiency anemia
• Platelet function is normal
Inherited Qualitative Disorders of Platelets
• Platelet function is impaired, usually secondary to an intrinsic defect of
platelets
• May be disorder of
• Adhesion
• Platelet release
• Platelet storage pool defects
• Disorders of adhesion
• Von Willebrand disease (v W D)
• Bernard-Soulier syndrome (B S S)
• Glanzmann’s thrombasthenia (G T)
Disorders of adhesion
von Willebrand Disease
• Autosomal dominant
• Pathophysiology: qualitative or quantitative defect in von Willebrand factor
• Symptoms: easy bruising, nosebleeds, heavy menses, excessive bleeding after tooth
extraction or dental procedures
• v W F is carried into peripheral circulation by Factor VIII
• v F W is an intermediary for platelet adhesion and is a receptor for G P I b, G P II b, and G P III a
• Three primary levels of v W D
• Type 1
• Type 2
• Type 3
• v W F protein is measured by
• Assessing role in adhesion
• Assessing secondary role in aggregation
• Assessing role in clotting factor activity
• Ristocetin cofactor activity is the best predictive assay (decreased)
BOX 16.2 Basic Test Descriptions for von Willebrand Disease
• Platelet count—measured by automated methods
• P T T—measures anticoagulant portion of factor VIII molecule
• F8:C—measures clotting portion of factor VIII
• v W F:R c o—measures function of v W F activity by ristocetin-induced platelet aggregation (R

I P A)
• v W F:A g—measures amount of circulating protein but does not capture function of the
protein
• Treatment is tailored to the type or subtype of v W D
• Desmopressin acetate (D D A V P)
• Injectable or nasal spray
• High purity Factor VIII products
Bernard-Soulier syndrome
• Autosomal recessive
• Rare adhesion defect of platelets involving G P I b–IX complex
• G P I b–IX complex is site for
• Thrombin binding
• Regulating platelet shape
• Regulating reactivity
Bernard-Soulier syndrome
• Clinical symptoms
• Epistaxis
• Gingival bleeding
• Menorrhagia
• Purpura
• Thrombocytopenia with giant platelets
• Ristocetin-induced platelet aggregation is absent
• Platelet aggregation with epinephrine, thrombin, and collagen is normal
• Treatment: platelet transfusion
Glanzmann’s Thrombasthenia
• Rare autosomal recessive disorder
• Symptoms homozygous
• Deficiency or abnormality of G P II b and III a
• Fibrinogen binding to platelets (platelet aggregation)
• Variable bleeding patterns
• Umbilical cord, gingival bleeding, purpura, prolonged bleeding from minor cuts
Glanzmann Thrombasthenia
• Clinical symptoms
• Normal platelet count
• Normal platelet morphology
• Abnormal aggregation with all aggregating agents
except ristocetin
• Treatments depend on severity of bleeding episode
• Oral contraceptives (menorrhagia), epsilon-aminocaproic acid (E AC A) as a
topical thrombin-inducing agent
Platelet Release Defects
• Inherited disorders
• Abnormal secondary phases of platelet aggregation
• Associated with postoperative bleeding combined with
menorrhagia and easy bruisability
• Platelet count may be normal
• Platelet release defects
• Hermansky-Pudlak syndrome
• Chediak-Higashi syndrome
• Wiskott-Aldrich syndrome
• Thrombocytopenia with absent radii (T A R)
• Gray platelet syndrome
• Hermansky-Pudlak syndrome
• Autosomal recessive
• Dense granules deficient
• Results in albinism and hemorrhagic events
• Chediak-Higashi syndrome
• Autosomal recessive disorder
• Results in albinism
• Giant lysosomal granules in neutrophils
• Frequent infections because of impaired phagocytic ability
• Platelet release impaired
• Hepatosplenomegaly
• Wiskott-Aldrich syndrome • Thrombocytopenia with absent radii (T A R)
• X-linked recessive disorder • Rare disorder
• Severe eczema • No radial bones
• Recurrent infections • Skeletal and cardiac abnormalities
• Immune defects • Thrombocytopenia
• Gray platelet syndrome
• Platelets lack alpha granules
• Platelets are gray or blue-gray color in P S
• Large platelets
• Bleeding tendencies
• Bruisability
Acquired Defects of Platelet Functions
• Extrinsic platelet abnormalities
• Drug-related platelet abnormalities
• Aspirin (most commonly used)
• Restrains C O X-1 inhibitors by blocking formation
of prostaglandin synthesis
• Inhibits production of platelet aggregator
thromboxane A2
• Effect lasts 7 to 10 days
• Preventive for strokes, heart attacks, or other
cardiovascular events
Acquired Defects of Platelet Functions
• Platelet disorder secondary to main disorder
• Renal disease
• Purpura, epistaxis, hemorrhage
• Uremia
• ↓ Thromboxane synthesis, ↓ adhesion, ↓ platelet release
• ↓ Aggregation
• Multiple myeloma and Waldenström’s macroglobulinemia
• Increased proteins cause abnormal participation in
the activation of coagulation factors and fibrin formation
• Post-op bleeding and ecchymoses
Vascular Disorders Leading to Platelet
Dysfunction
• Abnormality of
• Skin
• Collagen
• Blood vessels
• Platelet function and platelet count are normal
Vascular Disorders Leading to Platelet
Dysfunction
• Causes • Purpura
• Mucosal bleeding • Due to Infectious agents
• Purpura • Amyloidosis
• Petechia • Vitamin C deficiency (scurvy)
• Ecchymosis • Cushing syndrome
• Telangiectasia

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Module I

Quantitative & Qualitative

• Infections with viral

• Spleen holds one-third of platelet volume

• Idiopathic thrombocytopenic purpura (I T P) Acute

• Thrombotic thrombocytopenic purpura (T T P)

• Hemolytic uremic syndrome (H U S)

• Platelet count—measured by automated methods

• P T T—measures anticoagulant portion of factor VIII molecule

• F8:C—measures clotting portion of factor VIII

• v W F:R c o—measures function of v W F activity by ristocetin-induced platelet aggregation (R

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