Information About

Hemophilia B




  OMIM 306900
  MedlinePlus 000539
  EMedicineSubj emerg
  EMedicineTopic 240
  DiseasesDB 5561
  MeshID D002836


Haemophilia B (also spelled '''Hemophilia B''' or '''Hæmophilia B''') is a 1952;2:1378-1382. PMID 12997790.


TREATMENT

Treatment (bleeding prophylaxis) is by intravenous infusion of factor IX.


GENETICS

The factor IX gene is located on the X Chromosome (Xq27.1-q27.2). It is inherited X-linked recessive, which explains why - as in haemophilia A - mostly males are generally affected.


PATHOPHYSIOLOGY

Factor IX deficiency leads to an increased propensity for Haemorrhage . This is in response to mild trauma or even spontaneously, such as in joints ( Haemarthrosis ) or muscles.


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