Peripheral T cell lymphoma after chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): a case report
Peripheral T cell lymphoma after chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): a case report
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DOI：
10.1186/s12883-019-1507-z
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发表时间：
2019-11-04
期刊：
影响因子：
2.6
通讯作者：
Zhang, Lu
中科院分区：
文献类型：
--
作者：
Liu, Xiao-hang;Jin, Fan;Zhang, Lu
背景 类固醇反应性慢性淋巴细胞炎症伴脑桥血管周围增强（CLIPPERS）是一种中枢神经系统（CNS）炎症性疾病，具有独特的临床、放射学和病理学特征。 CLIPPERS 的病理生理学仍不清楚。由于有少数类似CLIPPERS表现的淋巴瘤病例报道，而且淋巴瘤的预后较差，因此淋巴瘤的早期识别非常重要。病例介绍 一名31岁女性因复视、头晕、步态共济失调、右侧面部麻木病史3个月入院。 CLIPPERS 的诊断是基于磁共振成像 (MRI) 中发现小脑、丘脑、脑桥、延髓和中脑区域的点状增强病变，以及对皮质类固醇的良好临床和放射学反应。然而，近10年后，她通过肺结节和皮肤活检被诊断为外周T细胞淋巴瘤，未另有明确（PTCL-NOS），化疗后1年内得到完全缓解。结论 我们报告了第一例发生 PTCL-NOS 的 CLIPPERS 病例。本病例提出，在难以进行脑活检时，在正电子发射断层扫描-计算机断层扫描（PET-CT）辅助检查下对脑外病灶进行活检有助于进一步鉴别。
Background Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disorder in the central nervous system (CNS) with distinct clinical, radiological, and pathological characteristics. The pathophysiology of CLIPPERS still remains unclear. Because a few cases about lymphoma mimicking the manifestations of CLIPPERS were reported and the prognosis of lymphoma is much worse, early identification of lymphoma is very important. Case presentation A 31-year-old woman was admitted with 3 months' history of diplopia, dizziness, gait ataxia, and right facial numbness. The diagnosis of CLIPPERS was established based on the finding of punctate enhancing lesions in the cerebellum, thalamus, pons, medulla, and midbrain region in magnetic resonance imaging (MRI), together with the favorable clinical and radiological responses to corticosteroids. However, she was diagnosed as peripheral T cell lymphoma, not otherwise specified (PTCL-NOS) by the pulmonary nodular and the skin biopsy almost 10 years later, and she got complete remission within 1 year after chemotherapy. Conclusion We report the first case of CLIPPERS developing PTCL-NOS. This case proposes that when brain biopsy was difficult to achieve, biopsies in extra-cerebral lesions under the assisting examination of positron emission tomography-computed tomography (PET-CT) can be helpful in further identification.