HEREDITARY BLEEDING DISORDERS IN RIYADH, SAUDI-ARABIA

The result of an eight-year retrospective analysis of patients with he reditary bleeding disorders (HBD) at King Khalid University Hospital, Riyadh, is presented. One hundred and sixty-eight patients referred fo r investigation for suspected bleeding disorders had bleeding symptoms which fulfilled the criteria for HBD and were categorized as follows: 1) coagulation factor deficiencies: 41 patients had hemophilia A, whi le 16 had hemophilia B; two patients each had factors XI and XII defic iency; four patients each had factors V and VIII deficiency and one pa tient had factor VII deficiency. There were two patients with dysfibri nogenemias and one with afibrinogenemia. 2) Von Willebrand's disease w as the second most common cause of HBD-25 patients were encountered in 15 different families. 3) Qualitative platelet disorders consisted of Glanzmann's thrombasthenia, with 18 patients, Bernard-Soulier disease , with five patients, and other qualitative platelet disorders, with 3 3 patients. 4) In 14 patients who presented with a history of bleeding , the only abnormality noted was prolongation of the bleeding time and normal coagulation and platelet function, and no definitive diagnoses could be established. The distribution of hereditary bleeding disorde rs obtained in this study resembles what has already been established in Western countries, with the exception of an increase of platelet di sorders, mostly due to the increased rate of consanguinity in the comm unity.