Blanco A, Palacio C, López A, Acebedo G, Castellá D, Juliá A
Unidad Clínica del Servicio de Hematología, Hospital General y Universitario Vall d'Hebron, Barcelona.
Med Clin (Barc). 1998 Oct 3;111(10):385-8.
Thrombotic Thrombocytopenic Purpura (TTP) is an uncommon clinical syndrome with high mortality rate in the absence of treatment. Despite the therapeutic efficacy of plasma exchange, patients often relapse even after long periods of time in remission. Over the last few years, late relapses in previously diagnosed patients have been seen in our hospital.
We analyzed retrospectively 22 episodes of TTP in 16 patients diagnosed during a four-year period. We reviewed the clinical features at diagnosis as well as the therapeutic results. In all but one, the treatment included daily plasma exchange. Other treatments, including vincristine, were also used in addition to plasma exchange, in 18 of 21 episodes.
A complete remission was obtained in eighty-two percent of the episodes treated by plasma exchange. The median number of plasma exchange to achieve a complete remission was 6. In 4 episodes, 20 or more plasma exchange were required before achieving a satisfactory response. A complete remission was obtained in 78% of episodes where vincristine was used, versus 84% response rate in episodes where vincristine was not used. In four patients the cause death was directly related to TTP, while a fifth patients died of progressive lymphoma without evidence of TTP. Five of the eleven surviving patients relapsed with a median time to relapse of 24.6 months. Relapsing episodes presented with a less severe clinical picture including less clear signs of microangiopathic hemolytic anemia (MAHA), when compared with the initial ones. All patients in relapse responded promptly to treatment. The variables analyzed failed to predict either the response to treatment or the probability of relapse.
The therapeutic efficacy of plasma exchange in the treatment of TTP has been demonstrated in our series as previously observed by many other groups. We have observed some slow responders where the prolongation of treatment by plasma exchange succeeded in achieving a complete remission. The use of vincristine did not show any therapeutical advantage in our experience.
血栓性血小板减少性紫癜（TTP）是一种罕见的临床综合征，若不治疗死亡率很高。尽管血浆置换有治疗效果，但患者即使在长时间缓解后仍常复发。在过去几年里，我院出现了先前确诊患者的晚期复发情况。
我们回顾性分析了在四年期间确诊的16例患者中的22次TTP发作。我们回顾了诊断时的临床特征以及治疗结果。除1例患者外，所有患者的治疗均包括每日血浆置换。在21次发作中的18次，除血浆置换外还使用了其他治疗方法，包括长春新碱。
通过血浆置换治疗的发作中有82%获得了完全缓解。实现完全缓解所需的血浆置换中位数为6次。在4次发作中，在获得满意反应前需要进行20次或更多次血浆置换。使用长春新碱的发作中有78%获得了完全缓解，未使用长春新碱的发作中缓解率为84%。4例患者的死亡直接与TTP相关，而第5例患者死于进行性淋巴瘤，无TTP证据。11例存活患者中有5例复发，复发的中位时间为24.6个月。与初始发作相比，复发发作的临床表现较轻，包括微血管病性溶血性贫血（MAHA）的体征不那么明显。所有复发患者对治疗反应迅速。所分析的变量未能预测治疗反应或复发概率。
正如许多其他研究组之前所观察到的，我们的系列研究证明了血浆置换在治疗TTP中的疗效。我们观察到一些反应缓慢的患者，通过延长血浆置换治疗成功实现了完全缓解。根据我们的经验，使用长春新碱未显示出任何治疗优势。
