Edel Elvira, Al-Ali Haifa Kathrin, Seeger Susanne, Kauschat Dörte, Matthes Gert
Institute of Transfusion Medicine, Germany.
Transfus Med Hemother. 2010 Feb;37(1):13-19. doi: 10.1159/000264996. Epub 2010 Jan 7.
Thrombotic thrombocytopenic purpura (TTP) is a rare clinical disorder which was associated with poor prognosis for a long time. The outcome has been improved by the consistent introduction of thera-peutic plasma exchange (TPE) as standard treatment of TTP. PATIENTS AND METHODS: We describe our experience in the use of solvent/detergent-treated plasma (SDP) for TPE in TTP. We retrospectively analyzed acute TTP epi-sodes in 8 patients (mean age = 27 years, range 18-44 years) treated with TPE using SDP with regard to tolerability and efficacy. RESULTS: All 8 patients were positive for anti-ADAMTS-13 antibody. Seven out of 8 had a se-vere ADAMTS-13 deficiency. All patients responded rapidly to SDP TPE with an increase in platelet count to above 150 x 10(9)/l. Hemolytic anemia disappeared over the treatment period. Approximately 2,000 l SDP were used for more than 500 treatments. Treatment with SDP was well tolerated; none of the patients experienced an adverse drug reaction after exposure to SDP. No major complications occurred even after multiple TPE. CONCLUSION: Our investigations suggest that TPE using SDP as replacement fluid is an effective treatment for TTP. The data described also indicate that SDP might offer the benefit of reducing adverse drug reactions.
血栓性血小板减少性紫癜（TTP）是一种罕见的临床疾病，长期以来其预后较差。随着治疗性血浆置换（TPE）作为TTP的标准治疗方法的持续引入，其预后得到了改善。
我们描述了使用溶剂/去污剂处理的血浆（SDP）进行TPE治疗TTP的经验。我们回顾性分析了8例接受SDP进行TPE治疗的急性TTP发作患者（平均年龄=27岁，范围18 - 44岁）的耐受性和疗效。
所有8例患者抗ADAMTS - 13抗体均为阳性。8例中有7例存在严重的ADAMTS - 13缺乏。所有患者对SDP TPE反应迅速，血小板计数增加至150×10⁹/L以上。溶血性贫血在治疗期间消失。约2000升SDP用于500多次治疗。SDP治疗耐受性良好；接触SDP后无患者出现药物不良反应。即使多次进行TPE也未发生重大并发症。
我们的研究表明，使用SDP作为置换液的TPE是治疗TTP的有效方法。所描述的数据还表明SDP可能具有减少药物不良反应的益处。
