Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy.
Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy.
J Thromb Haemost. 2019 Mar;17(3):492-498. doi: 10.1111/jth.14381. Epub 2019 Feb 25.
Essentials Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease. Surgery is a possible trigger of acute TTP episodes and no guidelines are available. Six patients with severe ADAMTS-13 deficiency during remission underwent elective surgery. Patients were prophylactically treated to restore ADAMTS-13 activity and no relapses occurred. SUMMARY: Background Severe ADAMTS-13 deficiency has been recognized as the main risk factor for recurrence of thrombotic thrombocytopenic purpura (TTP). Several conditions, including surgery, may influence the levels of ultra-large von Willebrand factor and ADAMTS-13, acting as a trigger for an acute TTP event. Objectives To report our experience of management of six patients with acquired TTP who underwent elective surgery after prophylactic treatment to restore ADAMTS-13 activity levels. Patients Six patients followed for acquired TTP with severe ADAMTS-13 deficiency during remission were candidates for seven elective surgeries (inguinal hernioplasty, cholecystectomy, laparoscopic hysterectomy, oophorectomy, parotidectomy and two total hip arthroplasties). Results Four patients were treated with prophylactic plasma exchange (PEX) therapy immediately before surgery. One patient was treated with PEX therapy before her first surgery and with preemptive rituximab once her second surgery was scheduled. Because rituximab increased ADAMTS-13 levels only partially, she required one PEX procedure the day before her second surgery. One patient was treated with azathioprine after rituximab failure, obtaining a progressive increase of ADAMTS-13 activity to more than 40%. This level allowed her to undergo total hip arthroplasty without additional treatment. All surgeries were successful and no complications or relapses occurred. Conclusions Six patients with acquired TTP underwent seven successful surgical procedures using prophylaxis to restore ADAMTS-13 activity. Further observational studies or randomized clinical trials are needed to confirm whether prophylactic PEX could be the key factor in preventing relapse.
原发性血栓性血小板减少性紫癜（TTP）是一种危及生命的疾病。手术可能是急性 TTP 发作的诱因，但目前尚无相关指南。六名缓解期严重 ADAMTS-13 缺乏的患者接受了择期手术。患者接受预防性治疗以恢复 ADAMTS-13 活性，且未发生复发。
背景严重的 ADAMTS-13 缺乏已被认为是血栓性血小板减少性紫癜（TTP）复发的主要危险因素。多种情况，包括手术，可能会影响超大血管性血友病因子和 ADAMTS-13 的水平，从而成为急性 TTP 事件的诱因。
目的报告六例获得性 TTP 患者的治疗经验，这些患者在缓解期存在严重的 ADAMTS-13 缺乏，在接受预防性治疗以恢复 ADAMTS-13 活性水平后接受了择期手术。
患者六例获得性 TTP 患者在缓解期存在严重 ADAMTS-13 缺乏，符合七种择期手术（腹股沟疝修补术、胆囊切除术、腹腔镜子宫切除术、卵巢切除术、腮腺切除术和两次全髋关节置换术）的条件。
结果四名患者在手术前立即接受预防性血浆置换（PEX）治疗。一名患者在第一次手术前接受 PEX 治疗，在第二次手术计划时接受了抢先使用利妥昔单抗治疗。由于利妥昔单抗仅部分增加 ADAMTS-13 水平，她在第二次手术前一天需要进行一次 PEX 治疗。一名患者在利妥昔单抗治疗失败后接受了硫唑嘌呤治疗，ADAMTS-13 活性逐渐增加到 40%以上。这一水平使她能够在无需额外治疗的情况下接受全髋关节置换术。所有手术均成功，无并发症或复发。
结论六例获得性 TTP 患者接受了七次成功的手术，采用预防措施恢复 ADAMTS-13 活性。需要进一步的观察性研究或随机临床试验来证实预防性 PEX 是否是预防复发的关键因素。
