伴有骨骼以外髓外浆细胞瘤的多发性骨髓瘤九例并文献复习

A report of 9 multiple myeloma patients with extramedullary and extraosseous plasmocytoma and literature review

目的总结伴有骨骼以外髓外浆细胞瘤的多发性骨髓瘤（MM）患者临床表现及其治疗反应，加深对该病的认识。方法对2001年1月至2007年6月初诊或病情进展过程中出现骨骼以外髓外浆细胞瘤的9例患者临床表现及治疗反应进行分析。根据欧洲骨髓移植疗效标准对患者的治疗反应进行判断。结果9例患者中位年龄55（48～66）岁，男女比为8：1。1例患者在初诊时即出现骨骼以外髓外浆细胞瘤，8例患者在病情进展时出现：患者通常多个部位受累，对常规化疗反应差。中位随访19（3～47）个月，8例患者从病情进展到出现骨骼以外髓外浆细胞瘤的中位时间为4．5个月。而7例患者从出现骨骼以外髓外浆细胞瘤到死亡的时间为2个月，中位OS为23个月。结论伴有骨骼以外髓外浆细胞瘤的MM较罕见。患者通常多部位受累，对常规化疗反应差，预后不良。需要探讨新的治疗策略。

Objective Extramedullary plasmocytoma at diagnose or during the course of multiple myeloma is rare. In order to know this entity better and explore new therapy for it, by combining with literature review, we conducted a retrospective study to describe the clinical and laboratory features of this entity and the outcome of these manifestation. Methods From January 2001 to July 2007, 123 multiple myeloma patients were treated in our hospital. We analysed the clinical features of patients with extramedullary plasmocytoma at diagnose or during the course of multiple myeloma. The response to therapy was evaluated according to the EBMT criteria. Results 9 eligible patients out of 123 with MM were retrieved from the hematology department of Beijing Chaoyang hospital. The median age was 55 （range：48-66） with a female/male ratio of 1/8. One patients was found to have Extramedullary and extraosseous tumor at the time of MM diagnose, and eight patients developed Extramedullary tumor during the course of the disease. Multiple sites were usually involved. Resistance to traditional chemotherapy was frequent. With a median follow-up of 19 months （range：3-47）, 2 patients are alive. Median time from progression of disease to diagnose of Extramedullary disease was 4 and a half months. The median interval from diagnosis of Extramedullary disease to death was 2 months and OS was 23 months. Conclusion Extramedullary plasmocytoma is a rare manifestation of MM, with a cumulative incidence of 7.3 % of MM in our department. Multiple sites are usually involved. The resistance to the traditional chemotherapy is frequent and the prognosis is very poor. The new therapy is necessarily explored.