Hemophilia in Jordan: a four-year prospective study

ABSTRACT

A four-year prospective study of inherited hemorrhagic syndromes in Jordan was done. A total of 100 patients from 41 families were found to have hemophilia A [67 patients], Christmas disease [32 patients] and factor XI deficiency [2 patients]. There were 10 deaths "14. 9%" related to hemophilia A, and four to Christmas disease [12. 9%]. Arthropathy was found in 38. 6% of patients with hemophilia A and 29. 6% of patients with Christmas disease. Of the hemophilia A, 34 patients [50.7%] were severe. Nineteen [28.4%] were moderate and 14 [20.9%] were mild. Of the Christmas disease 13 patients [41.9%] were severe, 16 patients [51.6%] were moderate and two patients [6.4%] were mild. The two patients with factor XI deficiency were mild. Family history was negative in 23% of patients with hemophilia A and in 16% of patients with Christmas disease. No factor inhibitors were detected in any patient. It is concluded that hemophilia A is most frequent type of hemophilia seen in Jordan. High proportion of hemophiliacs were severe and a significant number of them had fatal hemorrhage. Hemophilic arthropathy was common. No family history of bleeding was found in a significant number of patients