Progress in hemophagocytic lymphohistiocytosis with central nervous system involvement
10.3760/cma.j.issn.1673-4408.2022.05.001
- VernacularTitle:噬血细胞综合征合并中枢神经系统受累研究进展
- Author:
Wenxin OU
1
;
Rui ZHANG
;
Tianyou WANG
Author Information
1. 国家儿童医学中心 首都医科大学附属北京儿童医院 血液病中心 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 100045
- Keywords:
Hemophagocytic lymphohistiocytosis;
Central nervous system;
Clinical manifestations;
Therapy
- From:
International Journal of Pediatrics
2022;49(5):289-293
- CountryChina
- Language:Chinese
-
Abstract:
Hemophagocytic lymphohistiocytosis is a systemic inflammatory state caused by abnormal activation and infiltration of lymphocytes and histocytes.Characteristic features include unremitting fever, hepatosplenomegaly, cytopenia, organ damage, and even failure.Patients can develop central nervous system involvement.The most common neurological symptoms of CNS involvement are seizures and mental status changes, which need to be distinguished from various CNS diseases, such as multiple sclerosis, acute disseminated encephalomyelitis, and encephalitis.Early diagnosis and treatment are essential because of the poor prognosis and permanent sequelae of children with CNS involvement.This article aims to review the mechanism, clinical manifestations, accessory examinations, diagnosis, differential diagnosis, and treatment of CNS lesions in hemophagocytic lymphohistiocytosis and find clues for improving the outcome.
