Rock G, Shumak K H, Sutton D M, Buskard N A, Nair R C
Department of Medicine, University of Ottawa, Ontario, Canada.
Br J Haematol. 1996 Aug;94(2):383-6. doi: 10.1046/j.1365-2141.1996.d01-1800.x.
The current established treatment of thrombotic thrombocytopenic purpura (TTP) is plasma exchange with fresh frozen plasma (FEP). With this treatment, there is a 49% response after seven exchanges and a 78% survival at 1 month. Although the exact cause of TTP is unknown, the presence of von Willebrand factor (VWF) multimers has been implicated in the disease. Accordingly, it has been suggested that cryosupernatant (plasma from which cryoprecipitate has been removed), which is relatively deficient in VWF multimers, might be an effective replacement fluid during plasma exchange. Patients from six centers were treated by plasma exchange with cryosupernatant. 18 patients who had failed a first course (average 7.7 exchanges) of plasma exchange with FFP. received a further seven exchanges with cryosupernatant. Subsequently, 40 previously untreated patients were exchanged with cryosupernatant. Of the 18 previously treated patients, 11 responded (defined as an increase in platelet count to > 150 x 10(9) /1 and no neurological events) after seven exchanges and 15 (83%) of the patients were alive at 1 month. The response rate in the 40 previously untreated patients was 75% at the end of seven exchanges and 95% of the patients were alive at 1 month. These values are significantly different (P < 0.05) from those reported in our earlier study and in other patients concurrently treated at the same centres with FFP when cryosupernatant was not available. Some patients who have failed to respond to plasma exchange with FFP replacement will respond to further exchange with cryosupernatant. Cryosupernatant replacement may be more effective as first-line treatment of TTP than FFP.
血栓性血小板减少性紫癜(TTP)目前公认的治疗方法是用新鲜冷冻血浆(FFP)进行血浆置换。采用这种治疗方法,7次置换后有49%的患者有反应,1个月时生存率为78%。虽然TTP的确切病因尚不清楚,但血管性血友病因子(VWF)多聚体的存在与该病有关。因此,有人提出,VWF多聚体相对缺乏的冷上清液(去除冷沉淀后的血浆)可能是血浆置换期间一种有效的替代液。来自6个中心的患者接受了用冷上清液进行的血浆置换治疗。18例首次用FFP进行血浆置换疗程(平均7.7次置换)失败的患者,又接受了7次冷上清液置换。随后,40例未经治疗的患者用冷上清液进行了置换。在18例先前接受过治疗的患者中,7次置换后有11例有反应(定义为血小板计数增加至>150×10⁹/L且无神经系统事件),15例(83%)患者在1个月时存活。40例先前未经治疗的患者在7次置换结束时反应率为75%,95%的患者在1个月时存活。这些数值与我们早期研究以及在同一中心同期用FFP治疗(当时没有冷上清液)的其他患者报告的数值有显著差异(P<0.05)。一些对用FFP替代进行血浆置换无反应的患者,对进一步用冷上清液置换会有反应。作为TTP的一线治疗,冷上清液置换可能比FFP更有效。