Collins Peter, Baudo Francesco, Huth-Kühne Angela, Ingerslev Jørgen, Kessler Craig M, Castellano Maria E Mingot, Shima Midori, St-Louis Jean, Lévesque Hervé
Arthur Bloom Hemophilia Centre, School of Medicine, Cardiff University, University Hospital of Wales, Cardiff, CF14 4XN, UK.
BMC Res Notes. 2010 Jun 7;3:161. doi: 10.1186/1756-0500-3-161.
Acquired hemophilia A (AHA) is a rare bleeding disorder caused by an autoantibody to coagulation factor (F) VIII. It is characterized by soft tissue bleeding in patients without a personal or family history of bleeding. Bleeding is variable, ranging from acute, life-threatening hemorrhage, with 9-22% mortality, to mild bleeding that requires no treatment. AHA usually presents to clinicians without prior experience of the disease, therefore diagnosis is frequently delayed and bleeds under treated.
Structured literature searches were used to support expert opinion in the development of recommendations for the management of patients with AHA.
Immediate consultation with a hemophilia center experienced in the management of inhibitors is essential to ensure accurate diagnosis and appropriate treatment. The laboratory finding of prolonged activated partial thromboplastin time with normal prothrombin time is typical of AHA, and the diagnosis should be considered even in the absence of bleeding. The FVIII level and autoantibody titer are not reliable predictors of bleeding risk or response to treatment. Most patients with AHA are elderly; comorbidities and underlying conditions found in 50% of patients often influence the clinical picture. Initial treatment involves the control of acute bleeding with bypassing agents. Immunosuppressive treatment to eradicate the FVIII inhibitor should be started as soon as the diagnosis is confirmed to reduce the time the patient is at risk of bleeding.
These recommendations aim to increase awareness of this disorder among clinicians in a wide range of specialties and provide practical advice on diagnosis and treatment.
获得性血友病A（AHA）是一种由针对凝血因子（F）VIII的自身抗体引起的罕见出血性疾病。其特征为无个人或家族出血史的患者出现软组织出血。出血情况各异，从急性、危及生命的大出血（死亡率为9%-22%）到无需治疗的轻度出血。AHA通常出现在临床医生面前，而这些医生此前并无该病的诊治经验，因此诊断常常延迟，出血也得不到充分治疗。
采用结构化文献检索来支持专家意见，以制定AHA患者管理的推荐方案。
立即咨询在抑制剂管理方面经验丰富的血友病中心对于确保准确诊断和恰当治疗至关重要。活化部分凝血活酶时间延长而凝血酶原时间正常这一实验室检查结果是AHA的典型表现，即便没有出血也应考虑诊断。FVIII水平和自身抗体滴度并非出血风险或治疗反应的可靠预测指标。大多数AHA患者为老年人；50%的患者存在的合并症和基础疾病常常影响临床表现。初始治疗包括使用旁路制剂控制急性出血。一旦确诊，应立即开始免疫抑制治疗以根除FVIII抑制剂，从而缩短患者的出血风险期。
这些推荐旨在提高广大专科临床医生对该疾病的认识，并提供有关诊断和治疗的实用建议。
