Acquired Qualitative Platelet Disorders

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Transcript 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
Any Questions?
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)
Thank You
for Your
Attention