CLIPPERS, a syndrome of lymphohistiocytic disorders
CLIPPERS, a syndrome of lymphohistiocytic disorders
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DOI：
10.1016/j.msard.2020.102063
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发表时间：
2020-07-01
影响因子：
4
通讯作者：
Huan Yang
中科院分区：
文献类型：
--
作者：
Zhibin Li;Zhiping Jiang;Huan Yang
背景资料：CLIPPERS（对类固醇有反应的伴有桥脑血管周围增强的慢性淋巴细胞性炎症）是一种慢性中枢神经系统（CNS）炎症性疾病。可能与淋巴瘤和巨噬细胞活化有关，而组织细胞（巨噬细胞）活化参与CLIPPERS发病机制的相关报道较少。我们介绍第一个？可能是剪刀？一位38岁男性患者，合并有组织细胞肉瘤（HS），发展为噬血细胞综合征（HPS）。病例报告：男，38岁，因面部麻木、复视、步态不稳及语言障碍29个月来就诊.脑部MRI显示钆增强，弥漫于脑桥并延伸至中脑、髓质、桥臂、小脑和丘脑。经糖皮质激素治疗后，患者的CNS症状明显改善，并伴有明显的放射学改善，但病程呈复发-缓解和糖皮质激素依赖性。在缓解期，腹部计算机断层扫描（CT）意外发现腹部多发性结节。HS通过腹部淋巴结活检的组织学检查诊断，伴有CLIPPERS复发，并最终进展为HPS。结论：CLIPPERS可能是一种淋巴组织细胞疾病综合征。
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 lym- phoma 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 acciden- tally 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.