A population based, unselected, consecutive cohort of patients with acquired haemophilia A
A population based, unselected, consecutive cohort of patients with acquired haemophilia A
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DOI：
10.1046/j.1365-2141.2003.04731.x
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发表时间：
2004-01-01
影响因子：
6.5
通讯作者：
Majer, R
中科院分区：
文献类型：
--
作者：
Collins, P;Macartney, N;Majer, R
既往获得性血友病A研究报告了转诊至专科中心的患者队列或专科中心经验的回顾性调查。这可能导致文献中的患者组比常规血液学实践中的患者组更严重。我们报告了一个连续的，在威尔士南部和西部的所有患者谁提出了获得性血友病A在1996年和2002年之间的队列。有18例患者，发生率为1.34/万/年。与先前报道的队列相比，我们的患者年龄较大，中位年龄为70岁，并且不太可能有基础诊断（27%）。出血表型不太严重，只有27%有危及生命或肢体的出血，41%不需要止血治疗。1例患者死于出血，3例死于与免疫抑制相关的并发症。与其他系列相比，对免疫抑制的反应较高，88%的治疗患者达到无法检测到的抑制剂和正常的因子VIII水平。这些数据表明，先前报告的队列可能代表更严重的受影响的患者，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，但它们可能并不普遍适用。
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.