CAJ | 학술논문

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.
목적 관찰붕체좌미연합지새미송치료원발성계통성(AL)정분양변성환자적료효화불량반응.방법 11례AL정분양변성환자중명학정분양물질자10례:κ형1례,λ형9례.면역고정전영양성자5례:IgG κ형1례,IgGλ형3례,IgAλ형1례.루급신장、간장、심장、장도중2개혹이상장기자10례.11례환자중초치자7례,난치자4례.채용붕체좌미연합지새미송방안치료.결과 8례가평개료효환자,중위료정3(1～6)개.중위수방시간6개월.기관공능개선자6례,중위기효시간2개월.3례병정진전,2례사망.상견적불량반응유복사、혈소판감소、주위신경병변、핍력、대상포진등.7례인불량반응감소료붕체좌미제량,기중2례최종중단치료.3례미완성1개료정치료,발생료전간、마비성장경조、좌심공능쇠갈、직립성저혈압등엄중불량반응.결론 붕체좌미치료AL정분양변성유효,불량반응발생솔고,부분교엄중.
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.