Coagulopathy, Factor IX Deficiency

Within USA, approximately 4000 persons are affected (20% of all hemophiliacs): incidence: 1:25,000–30,000 males; 75–100 are people born with the disease in USA each year.

No racial prevalence.

Highest prevalence overwhelmingly in males.

Increased risk of hemorrhagic complications from any procedure.

Of affected individuals, 60% have severe disease (<1% normal circulating factor IX).

Of carrier females, 10% have abnormal hemorrhage risk.

Excessive and/or uncontrollable hemorrhage

Tendency for recurrent hemorrhage after initial control

Expansive deep and soft-tissue hematomas

Increased risk if hepatic dysfunction from prior plasma product transfusions

Inherited; also called hemophilia B or Christmas disease.

Very similar to hemophilia A (classic hemophilia), but with somewhat less severe bleeding frequency and severity.

Hemarthrosis accounts for 75% of bleeding episodes. Chronic debilitating arthritis is a common development.

Soft-tissue hematomas and hematuria also common.

Intracranial hemorrhage is most common fatal complication.

Disease severity proportional to circulating factor IX activity (<1% normal activity = severe disease; >5% = generally mild disease).

Modern maintenance factor replacement treatment results in normal life expectancy.