儿童噬血细胞综合征的中枢神经系统表现

The Central Nervous Cystem(CNS) Manifestations of Hemophagocytic Syndrome in Children

噬血细胞综合征(HPS)又称噬血细胞性淋巴组织细胞增多症(HLH),是一种由遗传或获得性免疫调节异常导致的致死性过度炎症反应综合征,包括一系列的临床特征、异常实验室检查及异常影像学表现,常伴有多器官损害。近年来,关于HLH累及中枢神经系统(CNS-HLH)的报道越来越多,中枢神经系统受累甚至可能成为其唯一表现,且中枢神经系统受累往往提示预后不佳。目前对中枢神经系统受累的影像学检查方法主要为磁共振平扫及增强,仅极少数文献报道有MRS及DWI检查,且其结果具有临床意义。本文将对其中枢神经系统受累的临床表现、实验室检查及影像学表现进行概述。

Hemophagocytic syndrome(HPS) also known as Hemophagocytic lymphohistocytosis(HLH) is a kind of fatal over-inflammatory response syndrome caused by abnormal genetic or acquired immune regulation, which includes a series of clinical features, abnormal laboratory examinations and abnormal imaging manifestations, and is often associated with multiple organs’ damage. In recent years, more and more reports have been published on the involvement of HLH in the central nervous system(CNSHLH). CNS involvement may even be its only manifestation, and often indicates a poor prognosis. At present, plain scan and enhancement MRI are the main imaging methods for CNS involvement. Few literatures have reported MRS and DWI examination, and the results have clinical significance. In this paper, the clinical, laboratory and imaging findings of CNS involvement are summarized.