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目的:研究和分析多发性骨髓瘤(MM)伴AL淀粉样变性的临床特征。方法:回顾性分析33例经病理活检明确的MM伴AL淀粉样变性患者的临床、实验室检查、治疗及预后情况。结果:33例MM合并AL淀粉样变性患者中常见受累脏器为肾(75.76%)、心脏(54.55%),M蛋白主要为IgGλ轻链型(36.36%)、IgAλ轻链型(30.30%)。33例MM伴AL淀粉样变性患者的中位随访时间32(2~94)个月,中位总生存时间(OS)为30(1~94)个月。根据患者受累器官个数分组,1个器官受累患者的OS为43个月,2个器官受累者的OS降至21个月,3个或以上器官受累的患者OS仅12个月(P<0.05)。有心脏累及的患者中位OS时间为15(1~26)个月,低于其他患者的中位OS时间40(13~94)个月(P<0.05)。15例采用硼替佐米联合治疗的有效率为53.33%,中位生存期为30(10~94)个月(P=0.023),优于其他治疗。结论:MM伴AL淀粉样变性少见,常见受累部位为肾脏及心脏。MM伴淀粉样变是预后不良的因素,MM伴AL淀粉样变性累及器官越多,预后越差,其中累及心脏者预后更差,接受新型治疗可提高疗效。
Objective: To study and analyze the clinical features of multiple myeloma (MM) with AL amyloidosis. Methods: A retrospective analysis of 33 cases of pathologically biopsied MM with AL amyloidosis in patients with clinical, laboratory examination, treatment and prognosis. Results: The common organs involved in MM and AL were amnestic (75.76%) and heart (54.55%). The M protein was mainly IgG λ light chain (36.36%), IgAλ light chain (30.30% . The median follow-up time was 32 (2-94) months in 33 MM with AL, and the median overall survival (OS) was 30 (1-94) months. According to the number of affected organs, the OS of one organ affected was 43 months. The OS of the two organs decreased to 21 months. The OS of patients with 3 or more organs was only 12 months (P <0.05) ). The median OS time in patients with cardiac involvement was 15 (1-26) months, which was lower than the median OS time of 40 (13-94) months (P <0.05) in other patients. The effective rate of 15 cases treated with bortezomib was 53.33%. The median survival time was 30 (10 ~ 94) months (P = 0.023), which was better than other treatments. Conclusion: MM with AL amyloidosis rare, the common site of involvement of the kidney and heart. MM associated with amyloidosis is a poor prognosis factor, MM with AL amyloidosis involving organs, the prognosis is worse, including the prognosis of the heart worse, receiving new treatment can improve the curative effect.