Coagulation factor defect and

inhibitors Coagulation factor defect

or abnormal function.

ADNAN ALI
EMT (KMU)
Coagulation factor defect and inhibitors Coagulation factor defect or
abnormal function.
Factor deficiencies
The most important congenital deficiency is Factor VIII deficiency:
Called Hemphilia A
Graded as severe, moderate, and mild depending on the coagulant activity of Factor
VIII
Inherited as sex-linked ressesive manner and occurs exclusively in males
 von Willebrand’s disease:
 Due to deficiency of vWF
 Characterized by defects in platelet adhesion
 Factor IX deficiency:
 Called Hemophilia B
 Sex linked recessive
 Occurs less frequently and milder in its clinical presentation than factor
VIII deficiency (Hemopilia A)
Abnormal coagulation factors function

Abnormality in function is seen in vit K deficiency. The binding of Ca++ to factors

II, VII, IX, X is required for normal clotting.
Without the attached Ca++, these fators will not bind to phospholipids and rate of
factor activation will be sharply decreased. To bind Ca++ they need
gamacarboxylation with vit K (to make II, VII, IX & X functional)

Vit K dependant factors are: Factors II, VII, IX, X, Protein C & protein S
Disturbance of the balance between promotors and inhibitors of coagulation due
to:

 Various disorders including bacterial, viral, rickettsial, infections

 Complication of pregnancy
 AML(acute myeloid leukemia)

 Tissue damage (shock, heat strock, burns)

 Hemolytic transfusion reactions
 Venome snake bites