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Isbandiyah,dr, SpPD
Definition of HEMOSTASIS
• The arrest of bleeding by repair of vessel wall
• Maintaining a balance
– Coagulation
– Fibrinolysis
• Hypocoagulation: excessive bleeding (inherited or
acquired)
• Hypercoagulation (thrombosis) inadequate activation
of the fibrinolytic system
HEMOSTATIC PROCESS
• Primary Hemostasis
– Blood vessel contraction
– Platelet Plug Formation
• Secondary Hemostasis
– Activation of Clotting Cascade
– Deposition & Stabilization of Fibrin
• Tertiary Hemostasis
– Dissolution of Fibrin Clot
– Dependent on Plasminogen Activation
Systems Involved in Hemostasis
• Vascular system
– Injured vessel initiates vasoconstriction
• Platelet System
– Injured vessel exposes collagen that initiates platelet
aggregation and help form plug
• Coagulation System
– protein factors of intrinsic and extrinsic pathways produce
a permanent fibrin plug
Hemostasis Lab Tests
•CBC-Plt
BV Injury •BT,(CT)
•PT
Tissue •PTT
Neural Platelet Factor
Adhesion
and
Activation
Primary hemostatic
Reduced
plug Fibrin Plt Study
Blood flow formation Morphology
Function
Antibody
Stable Hemostatic Plug
Primary Hemostasis Secondary Hemostasis
• Pathogenesis
– Accelerated destruction of Ab-sensitized platelets by phagocytic
cells (especially in the spleen)
– Gp IIb-IIIa: most common auto Ag
• Classification:
– Acute ITP : generally in children, preceding viral infection
– Chronic ITP: in adult (80%), thrombocytopenia > 6 months
• Management of ITP
– Splenectomy
• Not always curative, but usually promotes long-term and sustained
responses
• Complete remission rate: 72%
• Major risk: fatal sepsis due to encapsulated organisms
– Vaccination
– Penicillin prophylaxis
14
von Willebrand Disease
• The most common inherited bleeding disorder
• About 1% of the population
• Types I, II, and III.
• Symptom & sign: easy bruising, epistaxis, gum bleeding, menorrhagia,
post-partum hemorrhage, post-surgical or dental extraction bleeding
• Lab findings:
– BT prolonged, PT normal,
– APTT normal or prolonged
Notes:
• FFP: Every ml contains 1 Unit of F.VIII
• Cryoprecipitate : Each bag contains 125 Units of F.VIII.
Vitamin K Deficiency
• Vitamin K is a fat-soluble vitamin that can be absorbed in the
presence of bile salts.
• Vitamin K is required for the production of coagulation factors
in the liver (factors II, VII, IX, X).
• Laboratory findings:
↓ platelets ,↓ fibrinogen, ↓ factors II, V, VII and ↑ FDP
• Treatment:
-Treatment underlying disease.
-Platelets and FFP transfusion.
-Heparin is restricted to widespread thrombosis e.g. purpura fulminans .
-Exchange transfusion with fresh blood in neonates.
Fibrinolysis
• Primary Fibrinolysis :
– excessive fibrinolysis caused by fibrinolytic enzyme (t-PA,
plasmin) in the circulation.
– e.g: thrombolytic treatment, APL (AML-M3), decreased
inhibitor (anti plasmin, PAI-1), Chronic liver disease