原发性轻链型淀粉样变诊治进展

Diagnosis and treatment progress of primary light-chain amyloidosis

原发性轻链型(AL型)淀粉样变是一种罕见的致死性浆细胞疾病。近年来,AL型淀粉样变的治疗已从硼替佐米时代迈入了达雷妥尤单抗的免疫治疗时代,但晚期患者的治疗选择、如何获得早期器官缓解、如何进行病情监测等问题仍有待进一步探索。2022年第64届美国血液学会年会上多篇报道聚焦于AL型淀粉样变的诊治进展,文章对此进行简要介绍。

Primary light-chain(AL)amyloidosis is a rare and fatal plasma cell disease.In recent years,the treatment of AL amyloidosis has changed from the era of bortezomib to the era of daratumumab immunotherapy.However,for the treatment choice of advanced-staged patients,how to achieve organ responses at the early stage and how to monitor the disease are questions that need to be further explored.The 64th American Society of Hematology Annual Meeting in 2022 has reported advances in the diagnosis and treatment of AL amyloidosis,which are briefly reviewed in this article.