Hemophilia B
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Coagulation
- Common Pathway of Coagulation
- Extrinsic Pathway of Coagulation
- Intrinsic Pathway of Coagulation
- Hemophilia A
- Hemophilia B
- Hemophilia C
- Factor V Leiden
- Antithrombin-3 Deficiency
Summary
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Key Points
- Hemophilia B
- Also known as Christmas Disease
- Pathogenesis
- Deficiency of Factor IX
- Leads to inadequate generation of thrombin (Factor IIa) and improper coagulation of blood
- Deficiency of Factor IX
- Genetics
- XLR inheritance
- Almost exclusively affects males
- XLR inheritance
- Presentation
- Clinical symptoms indistinguishable from other hemophilias
- Bleeding
- Hemarthrosis - bleeding into joints
- Intramuscular hemorrhage
- Easy bruising
- Bleeding after trauma or surgery (e.g. dental procedure)
- Diagnosis
- Definitive: Factor IX levels
- Elevated PTT
- Due to inhibited intrinsic coagulation pathway
- Normal PT
- Extrinsic (tissue factor) pathway is unaffected
- Normal Bleeding Time
- Platelet plug formation (primary hemostasis is unaffected)
- Treatment
- Recombinant Factor IX concentrate