CLIPPERS, a syndrome of lymphohistiocytic disorders

Abstract

Background: CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) is a chronic central nervous system (CNS) inflammatory disorder. It may be associated with lymphoma and macrophage activation, while the related report of histiocytes (macrophage) activation involved in pathogenesis of CLIPPERS is rare. We present the first "probable CLIPPERS" case associated with histiocytic sarcoma (HS) progressed to hemophagocytic syndrome (HPS) in a 38-year-old man patient.

Case presentation: The 38-year-old man presented with facial numbness, diplopia, gait ataxia and glossolalia for 29 months. Brain MRI showed gadolinium enhancement peppering the pons and extending into the midbrain, medulla, brachium pontis, cerebellum and thalamus. The patient's CNS symptoms were improved significantly and accompanied by marked radiological improvement after glucocorticoids therapy, while the disease courses presented relapsing-remitting and glucocorticoids-dependent. Multiple nodules in the abdomen were accidentally discovered by the abdominal Computed tomography (CT) during the remission period. HS was diagnosed by histological examination of the abdominal node biopsy accompanied by CLIPPERS relapse, and eventually progressed to HPS.

Conclusions: CLIPPERS could be a syndrome of lymphohistiocytic disorders.

Keywords: CLIPPERS; Hemophagocytic syndrome; Histiocytic sarcoma; Lymphohistiocytic disorders.