Abstract:

Objective  To retrospectively analyze the literature and summary the clinical features of adults with  hemophagocytic lymphohistiocytosis (HLH) and combined with central nervous system (CNS) involvement. Methods  Reports of adults with HLH and combined with CNS involvement from January 1950 to December 2014 were retrieved from databases of Wanfang, VIP, China National Knowledge Infrastructure (CNKI), PubMed, and Ovid and systematically analyzed. Results  A total of 21 cases of adults with HLH and combined with CNS involvement were collected. Their clinical manifestations varied and the most common ones were altered consciousness (66.7%), epilepsy (33.3%), dysarthria (19.0%), and ataxia (19.0%). The image changes were not specific and the most frequently involved areas were deep brain white matter (53.3%), followed by brain stem (40.0%) and cerebellum (26.7%) and some cases showed enhancement of lesions by enhanced scan. The features of cerebrospinal fluid were mild to moderate pleocytosis, different degrees of elevated protein content, and normal glucose level. The HLH secondary to autoimmune diseases was more likely to involve the CNS (38.1%) and the total mortality was 81.0%. Conclusion  Adults with HLH and involved CNS have high mortality and poor prognosis. Early diagnosis and treatment and prevention of CNS infiltration are key factors.

Key words: adult, hemophagocytic lymphohistiocytosis, hemophagocytic syndrome, central nervous system, clinical features, systems analysis