Registry of hemophilia and other bleeding disorders in Syria.
Creating a national registry for bleeding disorders is a major step in establishing a National Hemophilia Care Program in all countries. Creating such a registry which would contain accurate and regularly updated data, including laboratory analysis confirmed by a reference laboratory established at the Syrian Hemophilia Society. Blood samples were drawn and analysed in the Society reference laboratory for the following screening tests: prothrombin time (PT), APTT and coagulation factor assays. Inhibitor detection and VWF RiCof were performed depending on the result of the screening tests. HBs Ag, anti-HCV, anti-HIV 1+2 and syphilis tests were also performed to detect transfusion transmitted agents (TTA). Diagnosis of the bleeding disorder type was confirmed for 760 of these cases. Among the 760 confirmed patients, 82.5% had haemophilia. Among these, 89.6%were haemophilia A; 10.4% were haemophilia B; 8.3% had VWD; 9.2% had other rare bleeding disorders as follows: 1.2% FVII deficiency, 0.7% FV deficiency, 1.8% F1 deficiency, 0.4% FX deficiency, 1.4% platelets dysfunctions (mainly Glanzmann Thrombasthenia) and 3.7% had combined FVIII and FV deficiency. Eighty (21.3%) cases of 375 screened for transfusion transmitted agents were positive for at least one infection: 0.5% were HBsAg positive, 19.7% were anti-HCV positive, 0.8% had combined HBsAg and anti-HCV positivity and 0.3% was anti-Syphilis positive. All patients were negative for HIV1 and HIV2. The preliminary data presented here follow known data on haemophilia A, haemophilia B and VWD disease. This registry will certainly help in improving haemophilia care in Syria.
Immuno-Hematology and Blood Transfusion Department, Faculty of Medicine, Damascus University, Damascus, Syria. t-ali@scs-net.org
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D000293:Adolescent; D000328:Adult; D025861:Blood Coagulation Disorders, Inherited / Q000175:diagnosis*; D002648:Child; D002675:Child, Preschool; D006467:Hemophilia A / Q000175:diagnosis; D002836:Hemophilia B / Q000175:diagnosis; D006509:Hepatitis B / Q000175:diagnosis; D006514:Hepatitis B Surface Antigens / Q000097:blood; D006526:Hepatitis C / Q000175:diagnosis; D018937:Hepatitis C Antibodies / Q000097:blood; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008875:Middle Aged; D010641:Phenotype; D012042:Registries / Q000706:statistics & numerical data*; D013587:Syphilis / Q000175:diagnosis; D013593:Syria; D055815:Young Adult; D014842:von Willebrand Diseases / Q000175:diagnosis
D006514:Hepatitis B Surface Antigens; D018937:Hepatitis C Antibodies