原发性巨球蛋白血症颅内浸润一例报告附文献复习

Intracerebral infiltration by monoclonal plasmacytoid ceils in Waldenstroem＇ s macroglobulinemia： a case report and review of the literature

目的总结原发性巨球蛋白血症颅内浸润的诊疗体会。方法对1例原发性巨球蛋白血症颅内浸润（Bing-Neel综合征）患者的临床资料进行分析，并进行相关文献复习，为其诊断和治疗提供思路。结果患者1年前诊断为原发性巨球蛋白血症，治疗中患者出现持续性头痛。颅脑MRI平扫＋增强：右侧额颞叶可见一团块状异常占位病变，他呈高信号，增强后病灶强化明显；手术切除肿瘤组织行病理检查示额叶小细胞恶性肿瘤浸润；免疫组化染色检查示淋巴-浆细胞瘤样细胞颅内侵犯。结论原发性巨球蛋白血症颅内浸润罕见，化疗疗效有限，理想的治疗应该是放疗联合化疗。

Objective To sum up the clinical experience of the diagnosis and treatment of intracerebral infiltration by monoclonal plasmacytoid cells in Waldenstroem＇ s macroglobulinemia （Bing-Neel syndrome）. Methods The clinical data of the diagnosis and treatment of a case of Bing-Neel syndrome was analyzed. Results A 56-year-old male was diagnosed as Waldenstroem＇ s macroglobulinemia one year ago, and presented with persistent headache during the treatment period. Magnetic resonance imaging showed a high intensity area on T2-weighed images in the right frontal lobe which was well enhanced by gadoliniumdiethylenetriaminepenta-acetic acid. Infiltration of neoplastic cells was confirmed by biopsy. Immunohistochemieal examination showed that mature plasmacytoid cells in the cerebral parenchyma were immunoglobulin M positive. Conclusion Infiltration in CNS （Bing-Neel syndrome） is uncommon in Waldenstroem＇ s macroglobnlinemia. As there is no effective therapy for this Bing-Neel syndrome, combination of radiation and chemotherapy shoued be considered for this situation.