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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
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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)
Clinical Manifestation of Primary Hemostasis Disorders: Ecchymosis: Mucosal Bleeding: Epistaxis Gingival bleeding Gastrointestinal bleeding Menorrhagia Hematuria • Petechiae • Purpura
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
Platelet Disorders: • Number: Decreased (Thrombocytopenia) Increased (Thrombocytosis/ Thrombocythemia) • Function: • Adhesion • Release reaction • Aggregation
Thrombocytopenia Etiology: • Deficient platelet production • Abnormal platelet distribution • Increased platelet destruction
Deficient Platelet Production • Myelophthisic (marrow infiltratif processes) • Aplasia • Ineffective erythropoiesis • Congenital Disoders
Abnormal Platelet Distribution • Hypersplenism (splenomegaly) • Hemangioma (Kasabach-Merrit syndrome)
Increased Platelet Destruction • Immune (primary) (ITP) • Immune (secondary) (SLE, Infection) • Microangiopathic thrombocytopenia (TTP, HUS, DIC) • Pregnancy-associated thrombocytopenia (HELLP syndrome)
Qualitative Platelet Disorders • Congenital: • Platelet Membrane Defects • Platelet Release (secretion) Defects • Platelet Coagulant Defects • Von Willebrand Disease 2. Acquired
Acquired Qualitative Platelet Disorders • 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. • Primary purpura (Senile Purpura) • Secondary Purpura (Allergic)