原发性巨球蛋白血症颅内浸润一例报告附文献复习

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,T2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.

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

Objective To sum up the clinical experience of the diagnosis and treatment of intracerebral infihration by monoclonal plasmacytoid cells in Waldenstrom'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 Waldenstrom'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 gadolinium.diethylenetriaminepenta-acetic acid.Infiltration of neoplastic cells was confirmed by biopsy.Immunohistochemical examination showed that mature plasmacytoid cells in the cerebral parenchyma were immunoglobulin M positive.Conclusion Infiltration in CNS(Bing-Neel syndrome)is uncommon in WaldenstrOm's macroglobulinemia.As there is no effective therapy for this Bing-Neel syndrome.combination of radiation and chemotherapy shoued be considered for this situation.