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 1º 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

Cryoprecipitate

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.