Evaluation of the Clinical Status of Patients with Inherited Bleeding Disorders in Diyala-Iraq

Abstract

Background: Inherited bleeding disorders are rare diseases that are both complicated and expensive to manage, they are classified into: coagulation factor disorders, vessel wall defect and platelet disorders. Objective: To show the pattern of Inherited bleeding disorders in Diyala-Iraq regarding frequency, distribution and complications. Patients and methods: A review of all patient's records with bleeding disorders in the hemophilia care center in Al-Battol Teaching Hospital in Baquba during the period from the 1st of September 2014 to the 28th of February 2015 were included. All patients with a history of bleeding tendency were tested to confirm the diagnosis. History and clinical findings were recorded. Laboratory analysis included prothrombin time, activated partial thromboplastin time, bleeding time, and fibrinogen assay. Patients with prolonged activated partial thromboplastin time were tested for factors VIII and IX. If factor VIII was low, von Willebrand factor: antigen and von Willebrand factor: ristocetin cofactor was performed. Platelet aggregation studies were done when there was isolated prolonged bleeding time. All patients were tested for viral infection such as hepatitis B,C and Human immunodeficiency virus infection, patients with hemophilia A and B were evaluated for inhibitors. Results: A total of 72 registered patients were reviewed in the hemophilia care center in Al-Battol teaching hospital. Inherited bleeding disorders identified in these patients include hemophilia A 57(79%), hemophilia B 5(7%), von Willebrand disease 5(7%), factor VII deficiency 3(4%); and platelet function defects 2 (3%). There were 68 (94%) males and 4 (6%) females. Hemarthrosis was the most frequent complaint in hemophilia A and B 41patient (66%) with knee hemarthrosis in 29 (71%) of them. Viral hepatitis C was positive in ten patients(14%), while hepatitis B positive in one patient(1.4%).The number of patients with inhibitors to Factor VIII was 3(5.3%)from total number of patients with hemophilia A. Conclusion: Hemophilia A, hemophilia B and vWD are the commonly encountered inherited bleeding disorders in our governorate followed by other recessively transmitted disorders. Hemarthrosis involving knee joint was the most common complication. Inhibitor was detected in a small number of patients.