Allogeneic hematopoietic stem cell transplantation-associated thrombotic microangiopathy:9 cases report of children and literature review
10.3760/cma.j.issn.2095-428X.2018.18.013
- VernacularTitle:儿童异基因造血干细胞移植相关性血栓性微血管病9例病例分析并文献复习
- Author:
Yixuan HE
1
;
Sidan LI
;
Runhui WU
;
Bin WANG
;
Guanghua ZHU
;
Jun YANG
;
Kai WANG
;
Yan YAN
;
Chenguang JIA
;
Maoquan QIN
Author Information
1. 100045北京,国家儿童医学中心,首都医科大学附属北京儿童医院血液肿瘤中心,儿童血液病与肿瘤分子分型北京市重点实验室,儿科学国家重点学科,儿科重大疾病研究教育部重点实验室
- Keywords:
Transplantation-associated thrombotic microangiopathy;
Hematopoietic stem cell transplantation;
Child;
Treatment
- From:
Chinese Journal of Applied Clinical Pediatrics
2018;33(18):1419-1422
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze the clinical characteristics,treatment and prognosis of allogeneic hematopoietic stem cell transplantation (allo-HSCT)-associated thrombotic microangiopathy (TA-TMA) in children.Methods The clinical information,treatment and prognosis of 9 cases with TA-TMA hospitalized following alloHSCT from January 2008 to November 2017 in Hematology Oncology Center,Beijing Children's Hospital,Capital Medical University were retrospectively analyzed.Results Of all the 283 allo-HSCT recipients,9 patients (3.2％) were diagnosed as TA-TMA.Among them,there were 5 male and 4 female,with a median age of 94 months (39-129 months).The median time to of diagnosis was 63 days (6-342 days) after HSCT.Additionally,the median platelet counts,hemoglobin and lactate dehydrogenase(LDH) levels were 44 × 109/L [(7-75) × 109/L],76 g/L (40-105 g/L) and 594 U/L(445-1 386 U/L).Neurological symptoms were found in 5 of the patients,4 had kidney involvement,and 6 had gastrointestinal involvement.The major treatment of TA-TMA was plasma exchange,Rituximab and defibrotide instead of the use of calcineurin inhibitors.Finally,4 patients achieved response after treatment,5 children died of ineffective treatment.Conclusion TA-TMA is a fatal complication after allo-HSCT.It can lead to multiorgan and multi-systems dysfunction.If there are more than 2 systems involved in TA-TMA,it suggests poor prognosis.The combined therapy is better than monotherapy,and the selective individual treatment of TA-TMA is essential.
