Sidhu Davinder, Snyder Edward L, Tormey Christopher A
Department of Laboratory Medicine, Yale University School of Medicine, New Haven, Connecticut, 06510.
Department of Pathology and Laboratory Medicine, University of Calgary, Calgary, Alberta, , T2N 1N4, Canada.
J Clin Apher. 2017 Jun;32(3):158-162. doi: 10.1002/jca.21475. Epub 2016 Jun 1.
Thrombotic thrombocytopenic purpura (TTP) is a rare but serious disease caused by autoantibody-mediated deficiency in von Willebrand factor (VWF) cleaving protease, ADAMTS-13. The primary acute treatment is therapeutic plasma exchange (TPE). However, some patients can develop allergic/anaphylactic reactions to the replacement (i.e., donor) plasma over time. Two potential treatment strategies for patients with TTP who demonstrate severe allergic reactions to plasma used for exchange were examined.
Two patients with TTP exacerbations who developed severe allergic reactions to donor plasma were identified. One patient's TPE was re-initiated with Octaplas, a lot-batched solvent and detergent treated, type-specific, pooled donor plasma product. The other patient was exchanged with primarily albumin, followed by slow incremental exposures to donor plasma to mitigate exposures and allergic risks. Both patients were assessed for anaphylaxis.
Both treatment strategies were successful in preventing any further clinically significant allergic/anaphylactic reactions and facilitated both patients' TTP remissions.
Based on our experience with two similar patients with TTP exacerbations and history of anaphylactic reactions to plasma during TPE, we have identified two possible treatment protocols to achieve remission in this clinical dilemma. Substituting Octaplas for standard plasma or, alternatively, using albumin with slowly increasing amounts of standard plasma may help to mitigate the risk of further anaphylactic adverse events. J. Clin. Apheresis 32:158-162, 2017. © 2016 Wiley Periodicals, Inc.
血栓性血小板减少性紫癜（TTP）是一种罕见但严重的疾病，由自身抗体介导的血管性血友病因子（VWF）裂解蛋白酶ADAMTS - 13缺乏引起。主要的急性治疗方法是治疗性血浆置换（TPE）。然而，随着时间的推移，一些患者可能会对置换（即供体）血浆产生过敏/过敏反应。研究了两种针对在血浆置换中对所用血浆表现出严重过敏反应的TTP患者的潜在治疗策略。
确定了两名TTP病情加重且对供体血浆产生严重过敏反应的患者。一名患者的TPE使用Octaplas重新开始，Octaplas是一种经过批量溶剂和去污剂处理、血型特异性、混合的供体血浆产品。另一名患者主要用白蛋白进行置换，随后缓慢增加对供体血浆的接触量，以减轻接触和过敏风险。评估了两名患者的过敏反应情况。
两种治疗策略均成功预防了任何进一步的具有临床意义的过敏/过敏反应，并促进了两名患者TTP的缓解。
基于我们对两名TTP病情加重且在TPE期间有血浆过敏反应病史的类似患者的经验，我们确定了两种可能的治疗方案，以解决这一临床困境并实现缓解。用Octaplas替代标准血浆，或者使用白蛋白并逐渐增加标准血浆的量，可能有助于降低进一步发生过敏不良事件的风险。《临床血液分离杂志》32:158 - 162，2017年。©2016威利期刊公司
