Highlights
- •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.
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
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)To read this article in full you will need to make a payment
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Article info
Publication history
Published online: March 20, 2020
Accepted:
March 19,
2020
Received in revised form:
March 15,
2020
Received:
February 23,
2020
Identification
Copyright
© 2020 Elsevier B.V. All rights reserved.
