- •A case of CLIPPERS associated with histiocytic sarcoma and hemophagocytic syndrome.
- •CLIPPERS, a possible syndrome of lymphohistiocytic disorders.
- •The relationship between CLIPPERS and macrophage activation related diseases needs to be explored.
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.
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.
CLIPPERS could be a syndrome of lymphohistiocytic disorders.
Abbreviations:CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids), CNS (Central nervous system), HS (Histiocytic sarcoma), HPS (Hemophagocytic syndrome), CT (Computed tomography), MAS (Macrophage activation syndrome), MRA (Magnetic resonance angiography), CSF (Cerebrospinal fluid), AQP4 (Aquaporin 4), NMO (Neuromyelitis optica), MBP (Myelin basic protein), MOG (Myelin oligodendrocyte glycoprotein), GFAP (Glial fibrillary acidic protein), EBV (Epstein-Barr virus), RSV (Respiratory syncytial virus), CMV (Cytomegalovirus), HSV (Herpes simplex virus), COXV (Coxsackievirus), ADV (Adenovirus), DCs (dendritic Cells), HLH (Hemophagocytic lymphohistiocytosis)
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Published online: March 20, 2020
Accepted: March 19, 2020
Received in revised form: March 15, 2020
Received: February 23, 2020
© 2020 Elsevier B.V. All rights reserved.