Inherited Bleeding Disorders in Pediatric Patients; experience of the national referral center in Iraq.

Abstract

Inherited bleeding disorders can lead to lifelong bleeding; they are mainly caused by quantitative or qualitative defect of coagulation factors, von Willebrand factor (VWF) or platelets. No published data are available about the different types of inherited bleeding disorders in Iraq. To describe types, severity and presentation of inherited bleeding disorders in pediatric patients in the major referral center in Iraq. This is a cohort prospective descriptive study conducted at the National Center of Hematology, a referral center for bleeding disorders in Baghdad-Iraq, from January 2015 to December 2019. One hundred ninety-one patients, aged 1day to 14years, with suspected inherited bleeding disorder are included in this study. Each patient was interviewed, accompanied by a chaperone, mostly parent(s), with revision of personal and familial bleeding history, conducting a brief medical examination, and withdrawing blood for complete blood count, peripheral blood film, bleeding time, PT and APTT. Further investigations including mixing studies, lupus anticoagulant, clotting factor activity, VWF:antigen (VWF:Ag), VWF: Ristocetin cofactor (VWF:RiCof) and platelet function tests using light transmission aggregometry were performed only if indicated. The mean ± 1SD of age of patients is 5.3 ± 3years, with a male:female ratio of 1.3:1. Family history of a similar bleeding disorder is recorded in 44.9% patients (P < 0.05). Consanguineous marriage was observed in 70.8% of the families (P < 0.001). The most prevalent inherited bleeding disorder is von Willebrand disease (VWD) (43.9%), of which type 3 is the most common (86.9%). Thrombasthenia is the second most prevalent (39.8%) inherited bleeding disorder; of these, the majority have Glanzmann's thrombasthenia (82.9%). Hemophilia A is found in 9.4% of patients. Type 3 VWD, Glanzmann's thrombasthenia and hemophilia A are the most common inherited bleeding disorders in the central part of Iraq, collectively they constitute >86% of patients. Consanguineous marriage should be discouraged in our society to decrease hereditary bleeding disorders. Also, there is a need to increase awareness and knowledge of bleeding disorders to improve early identification, mitigate the risk of further bleeding and prevent complications.