Guide To The Types of Hemophilia

Parahemophilia

Also known as Owren's disease, parahemophilia was first identified in the 1940s. It is characterized by a deficiency in clotting factor V, a protein that speeds up the initial stage of clot formation. Parahemophilia affects both sexes equally, and the prevalence of the condition is approximately one in one million. So far, fewer than two hundred cases of parahemophilia have been reported around the world. Patients with factor V deficiencies typically experience easy bruising and frequent bleeding from the nose, mouth, and gums. In adults with severe parahemophilia, internal bleeding might develop in the lungs and the gastrointestinal tract, and this may become potentially life-threatening. Infants with parahemophilia have an elevated risk of intracranial hemorrhage (bleeding within the brain). To diagnose parahemophilia, doctors carry out a factor V assay test, and tests to measure thrombin clotting time, prothrombin time, and activated partial thromboplastin time may also be performed. Currently, fresh frozen plasma is the only available treatment for parahemophilia, and doctors typically prefer to use solvent-detergent fresh frozen plasma since it contains the most reliable level of factor V. In severe cases that involve acute bleeding, patients might receive platelet concentrates as well.

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