Progress in hemophagocytic lymphohistiocytosis with central nervous system involvement / 国际儿科学杂志

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.