Inherited bleeding disorders: a 14-year retrospective study.
Congenital bleeding disorders comprise a heterogeneous group of diseases that reflect abnormalities of blood vessels, coagulation proteins, and platelets. A 14-year retrospective study (1991-2005) was conducted for patients referred to the coagulation section of the Hematology Department (King Hussein Medical Center, Amman, Jordan), who had suffered from bleeding tendencies to assess the prevalence of bleeding disorders among Jordanians and to describe their clinical manifestations. Four hundred and three patients matched our criteria. All patients were screened with routine coagulation assays and a complete blood cell count; a factor assay was performed if indicated by the results of the screening assays. A total of 168 patients (41.6%) were diagnosed with a bleeding disorder caused by a factor deficiency, of which 17.1% were described as hemophilia A (n=69), 6.2% were described as vWD (n=25), and 4.2% were described as hemophilia B (n=17). A subset of the total patient population comprising 14.1% of the patients were diagnosed with a Rare Inherited Coagulation Deficiency (RICD), where 4.0% were FX deficient (n=16), 3.7% were FVII deficient (n=15), 3.7% were FV deficient (n=15), 2.5% were FXI deficient (n=10), and 0.2% were diagnosed with afibrinogenemia (n=1).
Eid
Suhair S
SS
Coagulation Section-Haematology Department, Princess Iman Research and Laboratory Sciences Center, King Hussein Medical Center, Amman, Jordan. sueeid@gmail.com
Kamal
Nazmi R
NR
Shubeilat
Taisir S
TS
Wael
Abu-Ghoush Mohammed
AG
D000293:Adolescent; D000328:Adult; D025861:Blood Coagulation Disorders, Inherited / Q000175:diagnosis* / Q000453:epidemiology*; D001779:Blood Coagulation Factors / Q000032:analysis; D001780:Blood Coagulation Tests; D002648:Child; D002675:Child, Preschool; D005260:Female; D006467:Hemophilia A / Q000175:diagnosis / Q000453:epidemiology; D002836:Hemophilia B / Q000175:diagnosis / Q000453:epidemiology; D006801:Humans; D007223:Infant; D007597:Jordan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D014842:von Willebrand Diseases / Q000175:diagnosis / Q000453:epidemiology
D001779:Blood Coagulation Factors