Overview
Definition
X-linked recessive bleeding disorder.
Epidemiology
Varies globally depending on genetics, environment, or exposure.
Etiology & Risk Factors
- Factor IX deficiency.
Clinical Symptoms
- Hemarthrosis
- deep muscle bleeding
- prolonged bleeding after surgery.
Clinical Approach
Diagnosis
- Prolonged aPTT
- Normal PT
- Low Factor IX assay.
Management
- Recombinant Factor IX replacement.
Complications
- Joint destruction
- Intracranial hemorrhage.