采用血浆置换后,死亡率从 > 90% 降低至 10%~30%。 在迅速确诊且尽快开始治疗时,预后最佳。 血浆置换在 3 周内对约 90% 的患者产生疗效,其中大部分反应发生在 10 天内。 尽管不能预测哪些患者会有反应,但无重度 von Willebrand 因子裂解酶 (ADAMTS-13) 缺乏症且无 ADAMTS-13 抑制剂的患者更可能对治疗有短暂或不完全的反应。[58]Mori Y, Wada H, Gabazza EC, et al. Predicting response to plasma exchange in patients with thrombotic thrombocytopenic purpura with measurement of vWF-cleaving protease activity. Transfusion. 2002;42:572-580.http://www.ncbi.nlm.nih.gov/pubmed/12084165?tool=bestpractice.com 重度 ADAMTS-13 缺乏症患者更可能出现复发型病程。[59]Zheng XL, Kaufman RM, Goodnough LT, et al. Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura. Blood. 2004;103:4043-4049.http://bloodjournal.hematologylibrary.org/content/103/11/4043.fullhttp://www.ncbi.nlm.nih.gov/pubmed/14982878?tool=bestpractice.com[60]Coppo P, Wolf M, Veyradier A, et al. Prognostic value of inhibitory anti-ADAMTS13 antibodies in adult-acquired thrombotic thrombocytopenic purpura. Br J Haematol. 2006;132:66.http://www.ncbi.nlm.nih.gov/pubmed/16371021?tool=bestpractice.com 昏迷是预后不良的体征。