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Therapeutic plasma exchange in patients with thrombotic thrombocytopenic purpura:Retrospective multicenter study
发布于：2013年4月22日 文字：【大】【中】【小】
Transfusion and Apheresis Science
Serdal Korkmaz, Muzaffer Keklik, Serdar Sivgin, Rahsan Yildirim, Anil Tombak, Mehmet Emin Kaya, Didar Yanardag Acik, Ramazan Esen, Sibel Kabukcu Hacioglu, Mehmet Sencan, Ilhami Kiki, Eyup Naci Tiftik, Irfan Kuku, Vahap Okan, Mehmet Yilmaz, Cengiz Demir, ?smail Sari, Fevzi Altuntas, Ali Unal ,Osman Ilhan
Abstract
Thrombotic thrombocytopenic purpura (TTP) is a particular form of thrombotic microangiopathy typically characterized by thrombocytopenia, microangiopathic hemolytic anemia, fever, neurological abnormalities, and renal dysfunction. TTP requires a rapid diagnosis and an adapted management in emergency. Daily sessions of therapeutic plasma exchange (TPE) remain the basis of management of TTP. Also, TTP is a rare disease that is fatal if it is not treated. TPE has resulted in excellent remission and survival rates in TTP patients.
Aim
We aimed to present our experience in 163 patients with TTP treated with TPE during the past 5 years from 10 centers of Turkey.
Patients and methods
One hundered and sixty-three patients with TTP treated with TPE during the past 5 years from 10 centers ofTurkeywere retrospectively evaluated. TPE was carried out 1–1.5 times plasma volume. Fresh frozen plasma (FFP) was used as the replacement fluid. TPE was performed daily until normalization of serum lactate dehydrogenase (LDH) and recovery of the platelet count to >150 × 109/dL. TPE was then slowly tapered. Clinical data, the number of TPE, other given therapy modalities, treatment outcomes, and TPE complications were recorded.
Results
Fifty-eight percent (95/163) of the patients were females. The median age of the patients was 42 years (range; 16–82). The median age of male patients was significantly higher than female (53 vs. 34 years; p < 0.001). All patients had thrombocytopenia and microangiopathic hemolytic anemia. At the same time, 82.8% (135/163) of patients had neurological abnormalities, 78.5% (128/163) of patients had renal dysfunction, and 89% (145/163) of patients had fever. Also, 10.4% (17/163) of patients had three of the five criteria, 10.4% (17/163) of patients had four of the five criteria, and 6.1% (10/163) of patients had all of the five criteria. Primary TTP comprised of 85.9% (140/163) of the patients and secondary TTP comprised of 14.1% (23/163) of the patients. Malignancy was the most common cause in secondary TTP. The median number of TPE was 13 (range; 1–80). The number of TPE was significantly higher in complete response (CR) patients (median 15.0 vs. 3.5; p < 0.001). CR was achieved in 85.3% (139/163) of the patients. Similar results were achieved with TPE in both primary and secondary TTP (85% vs. 87%, respectively; p = 0.806). There was no advantage of TPE + prednisolone compared to TPE alone in terms of CR rates (82.1% vs. 76.7%; p = 0.746). CR was not achieved in 14.7% (24/163) of the patients and these patients died of TTP related causes. There were no statistical differences in terms of mortality rate between patients with secondary and primary TTP [15% (21/140) vs. 13% (3/23); p = 0.806]. But, we obtained significant statistical differences in terms of mortality rate between patients on TPE alone and TPE + prednisolone [14% (12/86) vs. 3% (2/67), p < 0.001].
Conclusions
TPE is an effective treatment for TTP and is associated with high CR rate in both primary and secondary TTP. Thrombocytopenia together with microangiopathic hemolytic anemia is mandatory for the diagnosis of TTP and if these two criteria met in a patient, TPE should be performed immediately
此报道为来自土耳其10个中心、回顾性研究，包括163例病人，为临床诊断TTP（必须存在微血管病溶血性贫血和PLT<150×109/L，伴或不伴中枢神经系统、肾脏损害、发热），但这些患者均未检测ADAMTS13活性及抗ADAMTS13抗体；患者均为获得性TTP，文章中将其分为了原发TTP和继发TTP（如自身免疫疾病，妊娠，骨髓移植，恶性肿瘤，药物，胰腺炎，HIV及其他感染）；这些患者中只要诊断了TTP，即尽早开始血浆置换，血浆置换开始为每天1次，置换量为1-1.5倍血浆量，均为新鲜冰冻血浆；LDH正常及PLT>150×109/L时开始逐渐减停血浆置换。对血浆置换反应不佳或病情恶化者，加用糖皮质激素（1-2mg/kg/d）；如果病人仍无好转，部分中心使用：增加血浆置换量或次数、长春新碱、环磷酰胺、或抗CD 20单抗。
结果：82.8%患者有神经系统异常，78.5%患者有肾功能不全；只有6.1%患者存在TTP五联征。中位血浆置换次数为13（范围1-80次）。85.3%患者完全缓解，且完全缓解与非完全缓解患者相比，血浆置换次数稍多（中位15.0 VS 3.5，P<0.001）。血浆置换+糖皮质激素与单纯血浆置换相比，完全缓解率无明显差别（82.1% VS 76.7%，P=0.746）。14.7%患者死亡，单纯血浆置换组比血浆置换+糖皮质激素组死亡率高（14% VS 3%，P<0.001）。此文章中提到，最常见的继发TTP是恶性肿瘤，恶性肿瘤对血浆置换反应差，但也能到71.4%。
结论：血浆置换对于原发和继发TTP均有很高的缓解率。TTP诊断必须有微血管病性溶血性贫血和血小板减少，而且只有上述2条就可以开始血浆置换。
http://www.sciencedirect.com/science/article/pii/S1473050213001018