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作者信息
Jabr Dorra, Hsasna Roua, Kefi Salma, Kharrat Rachid, Ben Neji Hend, Ben Lakhal Raihane
机构信息
Clinical Hematology Department, Aziza Othmana Hospital, Faculty of Medicine of Tunis, University of Tunis el Manar, Tunis, Tunisia.
出版信息
Tunis Med. 2024 Jun 5;102(6):343-347. doi: 10.62438/tunismed.v102i6.4614.
INTRODUCTION
Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially fatal hematological disorder that requires urgent treatment. Once the diagnosis has been made, plasma exchange (PE) must be started immediately and until a response is obtained.
AIM
Evaluate PE in terms of responses and complications in the treatment of TTP.
METHODS
This was a monocentric, descriptive, retrospective study including patients in whom TTP was diagnosed and treated with plasmapheresis in the clinical hematology department at Aziza Othmana Hospital, between January 2010 and December 2020.
RESULTS
Our study included 26 patients. PE was initiated within a median of 1 day. The rhythm of exchanges was daily in 22 patients. Twenty PE-related complications were noted, hypocalcemia being the most frequent (30%). CR was achieved in 15 patients after PE alone. Nine patients were refractory, and six received 2nd-line treatment, with CR achieved in five patients. Relapse was noted in six patients (40%). They were treated by PE and only one patient received rituximab. Four patients had a response. The overall response rate was 69% and overall mortality was 30%. OS at 2 years was 68,3% and RFS was 84,4%. Factors associated with the achievement of CR were the fall in LDH at D5 of treatment (p=0,027,OR=0,59 ;IC 95%[0,32-1,08]) and the daily rhythm of PE (p=0,005, OR=0,35; IC 95%[0,14-0,91]).
CONCLUSION
Our results were comparable to those of the literature, but the rate of refractory disease was higher. Rituximab may enhance our results.
引言
血栓性血小板减少性紫癜（TTP）是一种罕见但可能致命的血液系统疾病，需要紧急治疗。一旦确诊，必须立即开始血浆置换（PE）并持续至获得反应。
目的
评估血浆置换在TTP治疗中的反应和并发症。
方法
这是一项单中心、描述性、回顾性研究，纳入了2010年1月至2020年12月期间在阿齐扎·奥斯曼纳医院临床血液科诊断为TTP并接受血浆置换治疗的患者。
结果
我们的研究包括26例患者。血浆置换的中位启动时间为1天。22例患者的置换节奏为每日一次。记录到20例与血浆置换相关的并发症，低钙血症最为常见（30%）。仅通过血浆置换，15例患者实现了完全缓解（CR）。9例患者难治，6例接受二线治疗，其中5例实现了CR。6例患者（40%）出现复发。他们接受了血浆置换治疗，只有1例患者接受了利妥昔单抗治疗。4例患者有反应。总体缓解率为69%，总死亡率为30%。2年总生存率为68.3%，无复发生存率为84.4%。与实现CR相关的因素包括治疗第5天时乳酸脱氢酶（LDH）下降（p = 0.027，OR = 0.59；95%置信区间[0.32 - 1.08]）以及血浆置换的每日节奏（p = 0.005，OR = 0.35；95%置信区间[0.14 - 0.91]）。
结论
我们的结果与文献报道相当，但难治性疾病的发生率更高。利妥昔单抗可能会改善我们的结果。
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