噬血细胞综合征合并中枢神经系统受累研究进展

Progress in hemophagocytic lymphohistiocytosis with central nervous system involvement

噬血细胞综合征是淋巴细胞和组织细胞异常活化和浸润引起的系统性炎症状态,以持续发热、肝脾肿大、血细胞减少、器官功能损害甚至衰竭为其临床特点。其常伴有中枢神经系统(central nervous system,CNS)受累。CNS受累的最常见症状有癫痫发作和意识状态改变,需要与一系列CNS疾病(如多发性硬化症、急性播散性脑脊髓炎和脑炎等)相鉴别。CNS受累患儿预后差,可能产生永久性后遗症,因此及时诊治很重要。该文对噬血细胞综合征患者CNS病变的发生机制、临床表现、辅助检查、诊断、鉴别诊断和治疗方法进行综述,为改善疾病预后提供理论依据。

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.