甲型血友病中抑制物的流行病学

Epidemiology of inhibitors in haemophilia A.

作者信息

Kreuz W, Escuriola-Ettingshausen C, Martinez-Saguer I, Güngör T, Kornhuber B

机构信息

Department of Haematology and Oncology, J.W. Goethe University Hospital, Frankfurt, Germany.

出版信息

Vox Sang. 1996;70 Suppl 1:2-8.

PMID:8869461

Abstract

One of the most serious complications of the treatment of haemophilia A is the development of inhibitors. Former studies mostly considered the prevalence of inhibitor development, thus underestimating its true risk. Prevalences ranged widely (7-18%) probably due to the populations studied and the study design. Recent prospective previously untreated patients (PUP) studies were more comparable because of similar study designs. Eight PUP studies regarding the incidence of factor VIII inhibitors were analyzed: The inhibitor incidences (Independent of severity of haemophilia) ranged from 18.4 to 28%. Evaluating only severe haemophiliacs (factor VIII < 2%) significantly higher incidences were found. After 9-36 exposure days (as medians inhibitor development occurred at 0.8-3.3 years of age (as medians).

摘要

治疗甲型血友病最严重的并发症之一是抑制物的产生。以往的研究大多关注抑制物产生的发生率，从而低估了其真正风险。发生率范围很广（7%-18%），这可能是由于所研究的人群和研究设计所致。近期针对既往未治疗患者（PUP）的前瞻性研究由于研究设计相似而更具可比性。分析了八项关于VIII因子抑制物发生率的PUP研究：抑制物发生率（与血友病严重程度无关）在18.4%至28%之间。仅评估重度血友病患者（VIII因子<2%）时，发现发生率显著更高。在9-36个暴露日之后（中位数），抑制物产生发生在0.8-3.3岁（中位数）。