Inherited Bleeding Disorders: A 14-year Retrospective Study

Suhair S Eid; Nazmi R Kamal; Taisir S Shubeilat; Abu-Ghoush Mohammed Wael

doi:10.29074/ascls.21.4.210

This article has a correction. Please see:

Errata - January 01, 2009

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Suhair S Eid, MSc⇑
1. is chief coagulation officer, Coagulation Section-Haematology Department, Princess Iman Research and Laboratory Sciences Center, King Hussein Medical Center, Amman, Jordan
Nazmi R Kamal, MD MJB FCAP
1. is head of the Haematology Department, Princess Iman Research and Laboratory Sciences Center, King Hussein Medical Center, Amman, Jordan
Taisir S Shubeilat, MD DCPath MJB
1. is consultant haematologist and head of the Princess Iman Research and Laboratory Sciences Center, King Hussein Medical Center, Amman, Jordan
Abu-Ghoush Mohammed Wael
1. is senior haematology officer of the Haematology Department, Princess Iman Research and Laboratory Sciences Center, King Hussein Medical Center, Amman, Jordan

Address for correspondence: Suhair S Eid, PO Box 143855 Amman, 11844, JORDAN. +962777466500, +9625854462 (fax).sueeid{at}gmail.com.

Extract

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).

Hemophilia A was the most prevalent disorder discovered among all bleeding disorders. Epistaxis was found to be the most frequent symptom among RICD patients. More hemostasis studies and surveys need to be undertaken in developing countries to estimate the relative numbers of all hemostasis abnormalities including factor deficiencies.

The spontaneous arrest of bleeding from a severed blood vessel is due to…

ABBREVIATIONS: aPTT = activated partial thromboplastin time; BT = bleeding time; CBC = complete blood count; KHMC = King Hussein Medical Center; PT = prothrombin time; RIPA = ristocetin induced platelet aggregation’ RICD = recessively inherited coagulation disorders; vWF = Von Willebrand Factor; vWF:Ag = von Willebrand factor antigen; vWF:Rcof = vWF ristocetin cofactor.