Spectrum of hemophilia in Diyala-Iraq

Abstract

Background: Deficiencies of coagulation factors have been recognized for centuries. The most common inherited factor deficiencies are the hemophilias, X-linked diseases caused by deficiency of factor VIII (hemophilia A) or factor IX ( hemophilia B). Objective: To determine the clinical status, complications and the treatment regimen used for the patients with hemophilia in Diyala province of Iraq. Patients and methods: A review of all patient's records with hemophilia in the hemophilia care center in Al-Battol teaching hospital in Baquba during the period from the1st January 2015-30th of June 2015 were included. The patient's records contain all the information required such as age, gender, address, age at diagnosis, severity, presenting complaint, viral status, presence of inhibitors and target joint involvement. All patients were tested to confirm the diagnosis, assess the severity, viral status and the presence of inhibitors by factor assay, activated partial thromboplastin time, prothrombin time, hepatitis B surface antigen, hepatitis C virus antibodies, Human immunodeficiency virus serology and mixing study. Results: A total of 64 registered patients were reviewed in the hemophilia care center in Al-Battol teaching hospital of them 59(92%) with hemophilia A, while the others 5(8%) with hemophilia B. All of them were male. With median age about 13 year and a range from 2 to 72 years. 20 patients(31%)with mild hemophilia ,11(17%)with moderate and 33(52%)with sever type. Positive family history documented in 42 patient(66%) .One patient (1.6%)had hepatitis B surface antigen positive test, with 9 patients (14.1%)with hepatitis C positive results, no patients with HIV positive tests.3 patient(11%)with sever hemophilia A had inhibitors.41 patient(64%)had target joint involvement. Conclusion: Hemophilia is a rare disease but imply heavy impact on the family and the society. The improvement in comprehensive care let those patients live longer with better quality of life.