Acquired Qualitative Platelet Disorders

Platelet Disorders Dr.Leni Lismayanti, SpPK Dept of Clinical Pathology RSHS/FKUP Bandung

Reference book: - Denise M Harmening. Clinical Hematology and Fundamental of Hemostasis. 5th edition. Philadelphia: FA Davis Co, 2009.

Introduction • Primary hemostasis  platelet + vessel • Disorders of primary hemostasis  inability to perform platelet plug. • Defect: quantitative & qualitative • Inherited, congenital, acquired • Clinical manifestation 

Hemostasis Primer (Sumbat trombosit)

Hemostasis Sekunder (Sumbat hemostasis) 4

Clinical Manifestation of Primary Hemostasis Disorders: Ecchymosis: • Petechiae • Purpura

Mucosal Bleeding: • Epistaxis • Gingival bleeding • Gastrointestinal bleeding • Menorrhagia • Hematuria

Laboratory Tests to Assess Disorders of Primary Hemostasis • Platelet count • Peripheral blood smear • Ivy bleeding time or PFA-100 • von Willebrand study • Platelet antibody testing

• Flowcytometry • Platelet aggregation study • BM aspiration & biopsy

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Platelet Disorders: 1. Number: Decreased (Thrombocytopenia) Increased (Thrombocytosis/ Thrombocythemia) 2. Function: • Adhesion • Release reaction • Aggregation

Thrombocytopenia Etiology: 1. Deficient platelet production 2. Abnormal platelet distribution 3. Increased platelet destruction

Deficient Platelet Production 1. 2. 3. 4.

Myelophthisic (marrow infiltratif processes) Aplasia Ineffective erythropoiesis Congenital Disoders

Abnormal Platelet Distribution 1. Hypersplenism (splenomegaly) 2. Hemangioma (Kasabach-Merrit syndrome)

Increased Platelet Destruction 1. Immune (primary) (ITP) 2. Immune (secondary) (SLE, Infection) 3. Microangiopathic thrombocytopenia (TTP, HUS, DIC) 4. Pregnancy-associated thrombocytopenia (HELLP syndrome)

Qualitative Platelet Disorders 1. Congenital: • Platelet Membrane Defects • Platelet Release (secretion) Defects • Platelet Coagulant Defects • Von Willebrand Disease 2. Acquired

Acquired Qualitative Platelet Disorders 1. 2. 3. 4. 5. 6. 7. 8.

Renal disease (Uremia) Liver disease Paraproteinemia Myeloproliferative diorders Acquired von Willebrand disease Cardiopulmonary bypass Acquired storage pool deficiencies Drug therapy

Vascular Disorders (purpura) Results from another disease process and is just one of the manifestations of the disease process. 1. Primary purpura (Senile Purpura) 2. Secondary Purpura (Allergic)

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