Department of Hematology, Hedi Chaker Hospital.
Laboratory of Hematology, Habib Bourguiba Hospital, University of Sfax, Tunisia.
Blood Coagul Fibrinolysis. 2022 Oct 1;33(7):418-421. doi: 10.1097/MBC.0000000000001156. Epub 2022 Jul 22.
Hemophilia is a rare constitutional hemorrhagic disorder. There is insufficient epidemiological data on hemophilia in Tunisia. To describe the epidemiological, clinical, therapeutic, and outcome of a cohort of patients with hemophilia in southern Tunisia. A retrospective study was conducted on patients with hemophilia at the Hemophilia Treatment Center of Southern Tunisia in Sfax over 38 years (from January 1982 to December 2020). Data were collected in a regional hemophilia registry of the South Tunisian center. We collected 141 cases of hemophilia, 85% of whom had hemophilia A and 15% had hemophilia B. The severe form represented 65%, followed by the moderate form at 25%. The prevalence of hemophilia was 4.4 in 100 000 population. Family history of hemophilia was found in 70%. The mean age of patients at diagnosis was 28 months. Hemophilia was detected in 87% of cases after hemorrhagic syndrome. Bleeding occurred mainly in hemarthrosis (73%), hematoma (70%), and visceral bleeding (28%). Intracranial bleeding occurred in 6% of cases. Thirty-six percent of patients were on prophylactic therapy. Hemophilic arthropathy was the most important orthopedic complication in our patients (38%). Inhibitory antibodies occurred in 16% of PWH. Transfusion-transmitted infections with HIV and hepatitis C were in 2 and 31% of cases, respectively. The prevalence of hemophilia is still underestimated in our center. The severe form of hemophilia is the most frequent. Hemophilic arthropathy was the most important complication in our patients. This showed that hemophilia is still a disabling disease in our country.
血友病是一种罕见的遗传性出血性疾病。突尼斯缺乏血友病的流行病学数据。描述突尼斯南部一个血友病患者队列的流行病学、临床、治疗和结局。对 38 年来(1982 年 1 月至 2020 年 12 月)在斯法克斯南部突尼斯血友病治疗中心的血友病患者进行了回顾性研究。数据是从南部突尼斯中心的区域血友病登记处收集的。我们共收集了 141 例血友病患者,其中 85%为血友病 A,15%为血友病 B。严重型占 65%,其次是中型占 25%。血友病的患病率为每 10 万人中有 4.4 例。70%的患者有血友病家族史。患者的诊断平均年龄为 28 个月。87%的病例在出血综合征后发现血友病。出血主要发生在关节积血(73%)、血肿(70%)和内脏出血(28%)。颅内出血发生在 6%的病例中。36%的患者接受预防性治疗。血友病性关节病是我们患者中最重要的骨科并发症(38%)。16%的 PWH 出现抑制性抗体。HIV 和丙型肝炎的输血传播感染分别占 2%和 31%。我们中心仍然低估了血友病的患病率。严重型血友病最常见。血友病性关节病是我们患者中最重要的并发症。这表明血友病在我国仍然是一种致残性疾病。