Horváth Orsolya, Prohászka Zoltán, Kállay Krisztián, Kassa Csaba, Stréhn Anita, Csordás Katalin, Sinkó János, Kriván Gergely
Doktori Iskola, Semmelweis Egyetem Budapest.
Gyermekhematológiai és Őssejt-transzplantációs Osztály, Egyesített Szent István és Szent László Kórház Budapest, Albert Flórián út 5-7. 11/4A, 1097.
Orv Hetil. 2017 Jul;158(27):1043-1050. doi: 10.1556/650.2017.30786.
Hematopoietic stem cell transplantation associated thrombotic microangiopathy is a multifactorial complication, and has variable incidence in study populations due to different diagnostic criteria. The diversity of activity parameters, like elevated laktát-dehidrogenáz, hematological parameters and kidney function are not specific variables after stem cell transplantation. Dysregulation of the classical and alternative pathway can play an important role in the pathomechanism of thrombotic microangiopathy, but the understanding of the role of complement activation under transplantation conditions requires further investigation. Monitoring of complement parameters, including terminal complement pathway activation complex during transplantation may help physicians to improve diagnostic strategy, to evaluate therapeutical options and to predict and follow up efficacy of complement blockade methods and supportive therapy. This review focuses on the development of diagnostic criteria and therapeutical options in thrombotic microangiopathy, and presents some preliminary findings while using different diagnostic criteria in pediatric patients. Orv Hetil. 2017; 158(27): 1043-1050.
造血干细胞移植相关血栓性微血管病是一种多因素并发症，由于诊断标准不同，在研究人群中的发病率也有所不同。像乳酸脱氢酶升高、血液学参数和肾功能等活性参数的多样性并非干细胞移植后的特异性变量。经典途径和替代途径的失调在血栓性微血管病的发病机制中可能起重要作用，但对移植条件下补体激活作用的理解仍需进一步研究。监测移植期间包括末端补体途径激活复合物在内的补体参数，可能有助于医生改进诊断策略、评估治疗方案以及预测和跟踪补体阻断方法和支持治疗的疗效。本综述重点关注血栓性微血管病诊断标准和治疗方案的发展，并展示了在儿科患者中使用不同诊断标准时的一些初步发现。《匈牙利医学周报》。2017年；158(27): 1043 - 1050。
