Rare Bleeding Disease in Sistan & Baluchestan

Rare Bleeding Disease in Sistan & BaluchestanRare Bleeding Disease in Sistan & Baluchestan

Sistan and Baluchestan Province has the highest number of people with Factor XIII deficiency in the world. Based on data recorded in the World Haemophilia database, one person per million has the disease, which causes a severe bleeding tendency, IRNA reported. But unfortunately in the southeastern province, 90 persons per million are affected by the disease, said Dr. Soheila Khosravi, general director of the provincial Blood Transfusion Organization. The high rate of consanguineous marriage is the reason for the disease.  Factor XIII protein stabilizes the formation of blood clots. Without it, clots will develop, but then break down and cause recurrent bleeding. Up to 30% of patients sustain spontaneous intracranial hemorrhage, a brain bleed, which is the leading cause of mortality. Other symptoms of FXIII deficiency include bruising and nose, mouth and muscle bleeds.