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HemophiliaDone By :
Sinan Abdulhammed
2Definition
bleeding disorders due to inherited deficiencies of coagulation factors
3Causes
Inherited as a sex linked recessive trait with bleeding manifestations only in males.female carriers transmit the abnormal gene. genes which control factor VIII and IX production are located on the x chromosome; if the gene is defective synthesis of these proteins is defective
4Types
Disease Factor deficiency
Haemophilia A VIII[8]
Haemophilia B IX[9]
Haemophilia C XI[11]
After injury, the initial hemostatic event is formation of the platelet plug, together with the generation of the fibrin clot that prevents further hemorrhage. In hemophilia A or B, clot formation is delayed and is not robust.
PATHOPHYSIOLOGY
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6PATHOPHYSIOLOG
Y
7Clinical Features
Bleeding in : ●Joints (Hem arthrosis) ● muscle or soft tissue ●Abdomen ●GI tract ●Intracranial*Serious* ●Around vital structures in the neck
8Severity
severity is dependent on blood levels of functioning factor VIII or IX
% normal factor level
Causes of bleeding
Severe < 1% bleeding after trivial injury or spontaneous
Moderate 1 - 5% bleeding after minor injury; occasional spontaneous bleeds
Mild 6 - 30 % following major trauma, surgical or dental procedures
9Diagnosis
● family history.
●measuring the level of factor activity in
the blood.
●prolongation of APTT.
●All other screening tests of with in normal
limit.
10Management
Replacement of missing clotting factor Plasma products Cryoprecipitate Concentrates of Factor VIII Factor IX.
Desmopressin Treatment of a Specific Bleeding Site
11Complication
Progressive destruction of a jointnerve compression, contractureICH & DeathDeafness
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