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Coagulation disorders.

Deficiencies of Clotting factors
Onset - delayed after trauma
Deep bleeding
Into joints - Hemarthroses
Into deep tissues – Hematoma
large skin bleed – Ecchymoses

Common disease :

Hemophilia A
Hemophilia B
Vitamin K deficency
Von Willebrand Disease – Coagulation + platelet

Factor VIII Deficiency:

Classic hemophilia (hemophilia A)
X-linked disorder (affects 1ยบ males)
Most common - severe bleeding
Severity of disease -less than 1,2- 5, 5- 60%
Abnormal aPTT – Intrinsic path.
Diagnosis - factor VIII assay
Treatment - factor VIII concentrate
Recombinant factor VIII

Factor IX Deficiency:

Christmas disease (Hemophilia B)
X-linked recessive disorder
Indistinguishable from classic hemophilia (F VIII)
Requires evaluation of factor VIII and IX activity levels to diagnose
Treatment - factor IX concentrate
Cryoprecipitate if factor IX unavailable

Acquired coagulation disorder:

Vitamin K deficiency
- neonates - decreased intestinal
flora and dietary intake
- oral anticoagulants
- fat malabsorption syndromes
Required for factors II, VII, IX, X
Prolonged PT and aPTT

Laboratory findings in coagulation disorder:

Normal bleeding time & Platelet count
Prolonged prothrombin time (PT)
deficiencies of II, V, VII, X
Prolonged time (aPTT)
all factors except VII, XIII.

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