How to Treat Involvement of the Central Nervous System in Hemophagocytic Lymphohistiocytosis?

ABSTRACT:

Opinion statement

Central nervous system (CNS)-hemophagocytic lymphohistiocytosis (HLH) is not a disease in itself, but it is part of a systemic immune response. The vast majority of patients with CNS-HLH also have systemic HLH and a large number of patients with primary and secondary HLH have CNS involvement. Reactivations within the CNS are frequent during the course of HLH treatment and may occur concomitant with or independent of systemic relapses. It is also important to consider primary HLH as an underlying cause of "unknown CNS inflammation" as these patients may present with only CNS disease. To initiate proper treatment, a correct diagnosis must be made. A careful review of the patient's history and a thorough neurological examination are essential. In addition to the bloo

SUBMITTER: Horne A 

PROVIDER: S-EPMC5290057 | biostudies-literature | 2017 Jan

REPOSITORIES: biostudies-literature