Collins Peter, Macartney Nicola, Davies Richard, Lees Steven, Giddings John, Majer Ray
Department of Haematology, University of Wales College of Medicine, Cardiff, UK.
Br J Haematol. 2004 Jan;124(1):86-90. doi: 10.1046/j.1365-2141.2003.04731.x.
Previous studies in acquired haemophilia A have reported on cohorts of patients referred to specialist centres or were retrospective surveys of specialist centre experience. This may have resulted in the literature representing a more severe group of patients than seen in routine haematological practice. We report on a consecutive, unselected cohort of all patients in south and west Wales who presented with acquired haemophilia A between 1996 and 2002. There were 18 patients, an incidence of 1.34/million/year. Compared with previously reported cohorts our patients were older, with a median age of 70 years, and less likely to have an underlying diagnosis (27%). The bleeding phenotype was less severe, with only 27% having life or limb threatening bleeds and 41% required no haemostatic treatment. One patient died of bleeding, but three died of complications related to immunosuppression. Response to immunosuppression was high compared with other series, with 88% of treated patients attaining an undetectable inhibitor and normal factor VIII level. These data suggest that previously reported cohorts may represent more severely affected patients and, whilst guidelines for treatment based on these assumptions may be valid for severely affected patients, they may not be universally applicable.
以往关于获得性血友病A的研究报告的是转诊至专科中心的患者队列，或是对专科中心经验的回顾性调查。这可能导致文献所呈现的患者群体比常规血液学实践中所见的更为严重。我们报告了1996年至2002年间在南威尔士和西威尔士出现获得性血友病A的所有患者的一个连续、未经挑选的队列。共有18例患者，发病率为每年1.34/百万。与先前报告的队列相比，我们的患者年龄更大，中位年龄为70岁，潜在诊断的可能性更小（27%）。出血表型较轻，只有27%的患者有危及生命或肢体的出血，41%的患者不需要止血治疗。1例患者死于出血，但3例死于与免疫抑制相关的并发症。与其他系列相比，免疫抑制的反应率较高，88%接受治疗的患者抑制剂水平检测不到且因子VIII水平正常。这些数据表明，先前报告的队列可能代表了受影响更严重的患者，虽然基于这些假设的治疗指南可能对严重受影响的患者有效，但可能并非普遍适用。
