造血干细胞移植后儿童肝窦阻塞综合征/肝小静脉闭塞症的研究新进展  

Advances in research of sinusoidal obstruction syndrome/veno-occlusive disease after hematopoietic stem cell transplantation in children

作　　者：周辰 陈静[1] Zhou Chen;Chen Jing(Department of Hematology/Oncology,Shanghai Children′s Medical Center,School of Medicine,Shanghai Jiao Tong University,Shanghai 200125,China)

机构地区：[1]上海交通大学医学院附属上海儿童医学中心血液肿瘤科,200125

出　　处：《国际输血及血液学杂志》2020年第4期306-312,共7页International Journal of Blood Transfusion and Hematology

基　　金：国家自然科学基金(81970114);上海交通大学医学院多中心临床研究课题(DLY201814)。

摘　　要：肝窦阻塞综合征/肝小静脉闭塞症(SOS/VOD),是一种造血干细胞移植(HSCT)后的早期并发症。儿童SOS/VOD在发病率、临床表现、诊断与治疗方面与成年人SOS/VOD存在诸多不同。在近几十年的研究基础上确立的新诊断标准与严重程度分级标准,为儿童SOS/VOD的预防、诊断和治疗提供了理论依据。进一步发现更多血清学及分子标志物,将有助于儿童SOS/VOD的早期诊断及治疗。以去纤苷为代表的药物治疗,可使SOS/VOD患儿的生存率增高。笔者拟从发病机制及高危因素、临床表现、辅助检查、诊断、鉴别诊断及治疗等方面,对近年来HSCT后儿童SOS/VOD的新进展进行阐述,以期为其诊治提供参考。Sinusoidal obstruction syndrome/veno-occlusive disease(SOS/VOD)is an early complication after hematopoietic stem cell transplantation(HSCT).SOS/VOD in children is different from adults in terms of morbidity,clinical manifestations,diagnostic criteria and treatment.The new diagnostic criteria and severity grading standards established on the basis of recent decades of research provide theoretical basis for the prevention,diagnosis and treatment of children with SOS/VOD.Further discovery of more serological and molecular markers is conducive to the early diagnosis and treatment of SOS/VOD in children.Drug treatment represented by defibrillin can improve the survival rate of children with SOS/VOD.This article reviews on the new progress of children with SOS/VOD after HSCT in recent years from the aspects of pathogenesis and high risk factors,clinical manifestations,assisted examination,diagnosis,differential diagnosis and treatments.

关 键 词：肝静脉阻塞性疾病 造血干细胞移植 诊断 儿童 肝窦阻塞综合征 肝小静脉闭塞症 

分 类 号：R726.5[医药卫生—儿科]