Stefanello Bianca, De Paula Erich Vinícius, Andrade Orsi Fernanda, Comenalli Marques Jose Francisco, Gasparotto Roveri Eduardo, Pereira Colella Marina, Castro Ozelo Margareth, Maria Annichino-Bizzacchi Joyce, Addas-Carvalho Marcelo
Hematology and Hemotherapy Center, University of Campinas, Campinas, São Paulo, Brazil.
J Clin Apher. 2014 Dec;29(6):311-5. doi: 10.1002/jca.21336. Epub 2014 Jun 3.
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy caused by decreased activity of ADAMTS13, resulting in reduced clearance of ultralarge von Willebrand factor (VWF) multimers. Treatment of TTP is therapeutic plasma exchange (TPE) with replacement with fresh frozen plasma (FFP). Cryoprecipitate-poor plasma (CPP) is a plasma product with lower concentrations of large VWF multimers, and similar amounts of ADAMTS13. CPP is regarded as at least as efficacious as FFP in TTP but evidence of additional benefits has not been demonstrated. Furthermore, there are limited data on the frequency of adverse events associated with CPP.
In our center, the choice between CPP and FFP is performed before the 1st TPE session at the physicians' discretion. Here, we retrospectively evaluated the efficacy and safety of CPP based on the number of sessions, volume of plasma exposure, frequency of exacerbations/relapses, and adverse events.
Fourteen patients with newly diagnosed TTP were included in this analysis. The proportion of CPP:FFP use was 5:9. There were no significant differences in age, gender, initial hemoglobin, platelet count, LDH, or etiology of TTP between groups. We observed a trend toward a higher number of TPE sessions and higher plasma exposure in CPP, compared to FFP-treated patients. Acute exacerbations were more frequent among patients treated with CPP (OR 26.6; 95%CI 1.01-703.51; P = 0.03). Mild allergic reactions were the most common treatment-related adverse event in both groups.
Our data suggest that CPP should not be used as 1st line treatment for newly diagnosed TTP patients.
血栓性血小板减少性紫癜（TTP）是一种由ADAMTS13活性降低引起的血栓性微血管病，导致超大血管性血友病因子（VWF）多聚体的清除减少。TTP的治疗方法是进行治疗性血浆置换（TPE），并用新鲜冷冻血浆（FFP）进行置换。少冷沉淀血浆（CPP）是一种血浆制品，其大VWF多聚体浓度较低，ADAMTS13含量相似。CPP在TTP治疗中被认为至少与FFP一样有效，但尚未证明有其他益处。此外，关于与CPP相关的不良事件发生频率的数据有限。
在我们中心，医生可自行决定在首次TPE治疗前选择使用CPP还是FFP。在此，我们根据治疗次数、血浆暴露量、病情加重/复发频率和不良事件，回顾性评估了CPP的疗效和安全性。
本分析纳入了14例新诊断的TTP患者。CPP与FFP的使用比例为5:9。两组患者在年龄、性别、初始血红蛋白、血小板计数、乳酸脱氢酶或TTP病因方面无显著差异。与接受FFP治疗的患者相比，我们观察到接受CPP治疗的患者TPE治疗次数更多、血浆暴露量更高的趋势。接受CPP治疗的患者急性加重更为频繁（比值比26.6；95%置信区间1.01 - 703.51；P = 0.03）。轻度过敏反应是两组中最常见的与治疗相关的不良事件。
我们的数据表明，CPP不应作为新诊断的TTP患者的一线治疗方法。
