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摩洛哥拉巴特血友病患者抗因子 VIII 和抗因子 IX 抗体的流行情况:单中心经验。

The prevalence of anti-factor VIII and anti-factor IX antibodies among patients with hemophilia in Rabat, Morocco: a single center experience.

机构信息

Department of Pediatrics, Cheikh Khalifa Ibn Zayed International University Hospital, Casablanca, Morocco.

Hemophilia Treatment Center and Bleeding Disorders, Hematology-Oncology Unit, Children's Hospital Rabat, Mohamed V University, Rabat, Morocco.

出版信息

Pan Afr Med J. 2022 Feb 14;41:126. doi: 10.11604/pamj.2022.41.126.29571. eCollection 2022.

Abstract

The emergence of anti-factor VIII and anti-factor IX antibodies in hemophilia A or B is the most serious complication of hemophilia. We aim to expose through a series of patient's data, collected between 02/2009 and 02/2016 in the pediatric service of university hospital of Rabat, Morocco, the epidemiological and clinical characteristics of these patients, and to highlight the therapeutic difficulties encountered during their treatment. Out of 120 hemophiliac patients, we included 22 hemophiliac patients (18.33%, p<0.004) who developed an antibody, 21 patients with hemophilia A. Among the patients, 54.5% (n=12) exhibited moderate hemophilia, while 45.5% (n=10) had major hemophilia. The average age at diagnosis is estimated to 12±6.6 years. The circumstances of diagnosis were dominated by therapeutic inefficiency (63.64% (n=14)), then came dental extraction (9.09% (n=2)), preoperative assessment 22.73% (n=5) and hemophiliac arthropathy in a single case. The titration of antibodies in a 12-person sample ranged from 0.6 UB to 84 UB, of which (41.67% (n=5)) were low responders. The therapeutic treatment was based on fresh frozen plasma (54.55% (n=7)), recombinant activated factor VII (18.2% (n=4)), recombinant activated factor VII and PFC (18.2% (n=4)), and induction of immune tolerance. The occurrence of an inhibitory antibody represents the major residual complication of replacement therapy.

摘要

在甲型或乙型血友病患者中出现抗凝血因子 VIII 或抗凝血因子 IX 抗体是血友病最严重的并发症。我们旨在通过一系列于 2009 年 2 月至 2016 年 2 月在摩洛哥拉巴特大学医院儿科病房收集的患者数据来揭示这些患者的流行病学和临床特征,并强调在治疗过程中遇到的治疗困难。在 120 名血友病患者中,我们纳入了 22 名(18.33%,p<0.004)出现抗体的血友病患者,其中 21 名患者患有甲型血友病。在这些患者中,54.5%(n=12)表现为中度血友病,而 45.5%(n=10)表现为重度血友病。平均诊断年龄估计为 12±6.6 岁。诊断的情况主要是治疗无效(63.64%(n=14)),然后是牙齿拔除(9.09%(n=2)),术前评估(22.73%(n=5))和单个病例的血友病性关节病。12 人样本中抗体的滴定范围为 0.6 UB 至 84 UB,其中(41.67%(n=5))为低反应者。治疗治疗基于新鲜冷冻血浆(54.55%(n=7)),重组活化凝血因子 VII(18.2%(n=4)),重组活化凝血因子 VII 和 PFC(18.2%(n=4))和免疫耐受诱导。抑制性抗体的发生是替代治疗的主要残留并发症。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7883/9011910/b0046b34fcfa/PAMJ-41-126-g001.jpg

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