What is Hemophilia B?

Hemophilia B
Hemophilia B. A chest radiograph at the time of the first admission showed the presence of diffuse ground-glass opacity in the bilateral lung fields. Repeated Diffuse Alveolar Hemorrhage in a Patient with Hemophilia B. Hajime K, Jiro T, Hiromasa H, Takashi U, Kato F Rintaro N, Tatsumi. Intern Med. (2017). Not Altered. CC.

Hemophilia B aka Christmas disease is a genetic disorder caused by defective or absent factor IX.

What is the Pathology of Hemophilia B?

The pathology of hemophilia B aka Christmas disease is:

-Etiology: The cause of hemophilia B aka Christmas disease is an inherited or acquired mutation in the factor IX gene, or acquired factor IX inhibitor.

-Genes involved: Factor IX gene.

-Pathogenesis: The sequence of events that lead to hemophilia B aka Christmas disease is factor IX deficiency or dysfunctional factor IX that results in prolonged bleeding.

How does Hemophilia B Present?

Patients with hemophilia B typically are males that present with excessive bleeding at a young age. Other symptoms, features, and clinical findings associated with hemophilia A include easy bruising, hemarthrosis, and epistasis.

How is Hemophilia B Diagnosed?

Hemophilia B is diagnosed with medical history, and clotting factor tests.

How is Hemophilia B Treated?

Hemophilia B aka Christmas disease is treated with a concentrated factor IX product when needed.

What is the Prognosis of Hemophilia B?

The prognosis of hemophilia B aka Christmas disease is good.