硼替佐米联合地塞米松治疗原发性系统性淀粉样变性  

Treatment of primary systemic amyloidosis with the combination of bortezomib and dexamethasone

作　　者：翟勇平[1] 刘海宁[1] 于亚平[1] 周晓钢[1] 宋萍[1] 李锋[1] 王学文[1] 

机构地区：[1]南京军区南京总医院血液科,210002

出　　处：《中华血液学杂志》2010年第5期319-322,共4页Chinese Journal of Hematology

基　　金：基金项目：南京军区医学科研项目（200716）

摘　　要：目的 观察硼替佐米联合地塞米松治疗原发性系统性（AL）淀粉样变性患者的疗效和不良反应.方法 11例AL淀粉样变性患者中明确淀粉样物质者10例：κ型1例,λ型9例.免疫固定电泳阳性者5例：IgG κ型1例,IgGλ型3例,IgAλ型1例.累及肾脏、肝脏、心脏、肠道中2个或以上脏器者10例.11例患者中初治者7例,难治者4例.采用硼替佐米联合地塞米松方案治疗.结果 8例可评价疗效患者,中位疗程3（1～6）个.中位随访时间6个月.器官功能改善者6例,中位起效时间2个月.3例病情进展,2例死亡.常见的不良反应有腹泻、血小板减少、周围神经病变、乏力、带状疱疹等.7例因不良反应减少了硼替佐米剂量,其中2例最终中断治疗.3例未完成1个疗程治疗,发生了癫痫、麻痹性肠梗阻、左心功能衰竭、直立性低血压等严重不良反应.结论 硼替佐米治疗AL淀粉样变性有效,不良反应发生率高,部分较严重.Objective To evaluate the efficacy and feasibility of bortezomib plus dexamethasone （BD） in patients with primary systemic （AL） amyloidosis. Methods Eleven AL amyloidosis patients, including four relapsed or progressed after previous therapies and 7 newly diagnosed were treated with BD. Ten patients had two or more organs involved. Precursor protein analysis showed that 1 was κ light chain, 9 λ light chain 5 patients with positive immunofixation including 1 IgG κ, 3 IgG λ and 1 IgA λ. BD was administered according to standard two-week schedule. Results Eight patients were evaluable, the median number of treatment cycles was 3 （range 1 -6）. Median follow-up duration was 6 months. At least one affected organ response was observed in six patients and median time to organ response was 2 months. Three patients progressed and two of them died. Toxicities were mainly diarrhea, thrombocytopenia, peripheral neuropathy, fatigue and herpes zoster, and 7 evaluable patients who had toxicities were adjusted dosage and 2 of them interrupted therapy. Epilepsia, paralytic ileus, acute cardiac dysfunction, and postural hypotention were occurred in 3 inevaluble patients. Conclusion Bortezomib plus dexamethasone is effective in AL amyloidosis. Adverse events are common, and in some patients are severe.

关 键 词：淀粉样变性 硼替佐米 地塞米松 

分 类 号：R597.2[医药卫生—内科学]