Primary Adult-Onset Hemophagocytic Lymphohistiocytosis with Neurologic Presentation

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare immune deregulatory disorder that predominantly presents in children. Here we describe three patients with adult-onset primary HLH whose initial presentations were characterized by neurological features, and we review the literature of published cases. These cases ranged in age from 17 to 30 and presented with a variety of neurological symptoms. One of our cases demonstrated numerous microhemorrhages on MR brain. This is the first published case of adult-onset HLH presenting with cerebral microhemorrhages. In addition, literature review identified five additional patients with isolated central nervous system presentation of primary HLH.

Keywords: Autoimmune disease; CNS inflammation; Genetics; Hemorrhage – cerebral; Immunology; Magnetic resonance imaging; Neuroimmunology; Neuroinflammation.