Allogeneic hematopoietic stem cell transplantation-associated thrombotic microangiopathy:9 cases report of children and literature review / 中华实用儿科临床杂志
Chinese Journal of Applied Clinical Pediatrics
; (24): 1419-1422, 2018.
Article
en Zh
| WPRIM
| ID: wpr-696608
Biblioteca responsable:
WPRO
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.
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Banco de datos:
WPRIM
Tipo de estudio:
Prognostic_studies
Idioma:
Zh
Revista:
Chinese Journal of Applied Clinical Pediatrics
Año:
2018
Tipo del documento:
Article