C0220 Incidence of hemophilia in the North of Tunisia

Abstract

Background: Hemophilia is a hereditary bleeding disorder characterized by a deficiency of factor VIII (hemophilia A) or factor IX (hemophilia B). It is established that hemophilia is a ubiquitous condition that affects one birth in 10,000, regardless of race or geographic region. We propose in this work to assess the incidence of hemophilia in the North of Tunisia with a literature review. The hemophilia treatment center at the hospital Aziza Othmana supports patients in northern Tunisia, which represents 60% of all tunisians cases. The estimated number of inhabitants is 5.19 million (50% of the country population). Methods: Assays of factors VIII and IX are performed by STA Compact, Stago®. Confirmation of the diagnosis ismade on two different samples. Results: Twenty cases of hemophilia were diagnosed between January 2010 and December 2011 in our center. The average of age was 3.37 years (2 months-28 years). The most frequent circumstances of diagnosis are hematomas and hemarthrosis following minor trauma (12 cases). Family history of hemophilia is found in 6 cases. For all patients, an isolated prolonged APTT with a correction to the mixture test was found. We note 15 cases of hemophilia A and 5 cases of hemophilia B. The distribution of cases according to severity is: severe (9 cases), moderate (8 cases) and minor (3 cases). Comment: The incidence of hemophilia on the north of Tunisia is 1.1cases per 10 000 births per year. The incidence found in our series corresponds to the incidence reported in the literature. However, the prevalence of hemophilia diagnosed in Tunisia remains low only 50% of the theoretical number of hemophilia is diagnosed.