摘要
目的探讨伴髓外浸润的多发性骨髓瘤(MM)的临床特点、治疗及预后。方法回顾性分析北京朝阳医院(西区)2005年5月至2010年12月收治的43例确诊为伴髓外浸润的MM患者的临床资料。在MM病程中出现髓外浸润者12例(第1组);在MM诊断时即有髓外浸润者31例(第2组)。结果患者中位年龄53(28~76)岁,男23例,女20例。IgG型15例,IgA型9例,IgD型2例,K轻链型6例,入轻链型6例,双克隆型3例,不分泌型2例。最常浸润的部位为皮肤及肌肉软组织(39例),其次为椎管内(18例)、深部软组织(16例)。第1组中9例以硼替佐米联合DECP(顺铂、足叶乙甙、环磷酰胺、泼尼松)方案化疗的患者,2例达到完全缓解(CR),4例达到部分缓解(PR),3例疾病进展死亡;2例采用传统化疗患者,均进展死亡;1例放弃治疗后死亡。第2组中11例患者采用传统化疗,7例达PR,4例死亡;20例采用以硼替佐米为主的联合化疗,12例达CR,7例达PR,1例死亡。中位随访时间为23(5~60)个月,至随访截止,存活17例,死亡26例。第1组患者的预计3年及5年总生存率分别为54.21%、27.10%,中位生存期36(10~120)个月;第2组患者的预计3年及4年总生存率分别为39.83%、13.28%,中位生存期23(5~52)个月。结论伴髓外浸润的MM患者临床表现复杂,治疗困难,预后差,尤其是诊断时即有髓外浸润者。采用以硼替佐米为主的联合化疗虽然可以取得较好的总反应率,但长期疗效仍不佳。
Objective To explore the clinical features, treatment and prognosis of muhiple myeloma (MM) with extramedullary plasmacytomas (EM). Methods A total of 43 patients were enrolled and divided into 2 groups. Group-1 had 12 patients of EM occurring after the diagnosis of MM while Group- 2 included 31 EM patients at the initial diagnosis of MM. Results The male-to-female proportion was 23:20 and there was a median age of 53 years. The distribution of different isotypes was IgG (n = 15 ) , IgA (n=9), IgD (n=2), K light chain (n=6), k light chain (n=6), biclonal myeloma (n=3) and nonsecretory myeloma (n = 2). The sites of complicating plasmacytoma included skin, muscle and spinal canal. Nine patients received bortezomib plus DECP (cisplatin, etoposide, cyclophosphamide and prednisone) and 2 patients underwent traditional chemotherapy in Group-1. The outcomes were as follows: complete remission ( CR, n = 2 ), partial remission ( PR, n = 4 ) and death ( n = 5 ). And 11 patients received traditional chemotherapy in Group-2, 7 attained PR and 4 died. Twenty patients received bortezomib plus other chemotherapeutic drugs in Group-2. The outcomes were as follows: CR (n = 12), PR ( n = 7) and death (n = 1 ). The median overall survival (OS) was 36 months (range : 10 - 120) in Group- 1 and 23 months ( range : 5 - 52) in Group-2 respectively. In Group-1, the estimated 3- and 5-year OS were 54. 21% and 27. 10% respectively. And in Group-2, the estimated 3- and 4-year OS were 39. 83% and 13.28% respectively. Conclusions The EM patients show aggressive, complicated and diverse clinical courses and the unusual manifestation of muhiple organ involvement by plasma cells. Traditional chemotherapy has a poor efficacy and the prognosis is unfavorable, especially for EM with concurrent MM. The combined treatment of bortezomib with second-line chemotherapy may achieve curative effects.
出处
《中华医学杂志》
CAS
CSCD
北大核心
2012年第12期838-841,共4页
National Medical Journal of China
关键词
多发性骨髓瘤
预后
髓外浸润
临床特征
化疗
Multiple myeloma
Prognosis
Extramedullary plasmacytomas
Clinical features
Chemotherapy