Bing-Neel综合征:1例报告及临床表现、诊断和治疗方案的系统回顾

Jaspreet S. Grewal , Preetkanwal K. Brar , Walter M. Sahijdak , Joseph A. Tworek , Elaine G. Chottiner
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引用次数: 23

摘要

bing - neel综合征是一种极其罕见的神经系统并发症Waldenström巨球蛋白血症(WM),首次描述于1936年。伴有或不伴有脑脊液(CSF)高球蛋白血症的肿瘤淋巴浆细胞样细胞和浆细胞浸润与中枢神经系统有关。病例报告我们报告一例69岁白人男性,10年WM病史。经核磁共振及脑脊液病理检查,诊断为Bing-Neel综合征。此外,对最新英语文献中报告的Bing-Neel综合征病例进行了全面的回顾。结果本例患者经颅放射治疗及鞘内化疗成功。在完成治疗后,他的临床和病理缓解期为3年。在文献回顾的基础上,我们也对Bing-Neel综合征的临床表现、诊断和治疗方案进行了总结和讨论。结论bing - neel综合征是一种罕见且可治疗的WM并发症。有神经系统症状的WM病史的患者应评估是否有Bing-Neel综合征。单独颅放射治疗或联合鞘内化疗比单独鞘内化疗更有可能获得持续的缓解。
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Bing-Neel Syndrome: A Case Report and Systematic Review of Clinical Manifestations, Diagnosis, and Treatment Options

Background

Bing-Neel syndrome is an extremely rare neurologic complication of Waldenström macroglobulinemia (WM) that was first described in 1936. It is associated with central nervous system infiltration by neoplastic lymphoplasmacytoid and plasma cells with or without cerebrospinal fluid (CSF) hyperglobulinemia.

Case Report

We report a case of a 69-year-old white man with a 10-year history of WM. He was diagnosed with Bing-Neel syndrome based on magnetic resonance imaging and pathology studies of CSF. In addition, a comprehensive review of the reported cases of Bing-Neel syndrome in the up-to-date English-language literature was performed.

Results

Our patient underwent successful treatment with cranial radiation and intrathecal chemotherapy. He has been in clinical and pathologic remission for 3 years following the completion of his treatment. Based on our literature review, we also summarize and discuss clinical manifestations, diagnosis, and treatment options for Bing-Neel syndrome.

Conclusion

Bing-Neel syndrome is a rare and potentially treatable complication of WM. Patients with a history of WM presenting with neurologic symptoms should be evaluated for possible Bing-Neel syndrome. Cranial radiation therapy alone or in combination with intrathecal chemotherapy is more likely to achieve sustainable remission than intrathecal chemotherapy alone.

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