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达雷妥尤单抗和塔非酰胺治疗并发免疫球蛋白轻链和转甲状腺素蛋白心脏淀粉样变性患者:一例报告

Concurrent Immunoglobulin Light Chain and Transthyretin Cardiac Amyloidosis Patient Treated With Daratumumab and Tafamidis: A Case Report.

作者信息

Nishihara Taiki, Kawano Yawara, Tasaki Masayoshi, Naito Ayuko, Yuki Hiromichi, Nagayoshi Yasuhiro, Takashio Seiji, Nishimura Nao, Ueda Mitsuharu, Tsujita Kenichi, Yasunaga Jun-Ichirou

机构信息

Department of Cardiovascular Medicine Amakusa Medical Center Amakusa Japan.

Department of Hematology Rheumatology, and Infectious Diseases Graduate School of Medical Sciences Kumamoto University Kumamoto Japan.

出版信息

EJHaem. 2025 Aug 13;6(4):e70122. doi: 10.1002/jha2.70122. eCollection 2025 Aug.

Abstract

Immunoglobulin light chain (AL) and wild-type transthyretin (ATTRwt) amyloidosis, while sharing similar clinical presentations, require distinct treatments. We report a rare case of a 75-year-old man with heart failure diagnosed with concurrent AL and ATTRwt cardiac amyloidosis. Immunohistochemistry and liquid chromatography-tandem mass spectrometry confirmed both AL and ATTR amyloid deposits in the heart. The patient was safely and effectively treated with daratumumab-based chemotherapy followed by tafamidis. This case underscores the necessity of precise amyloid typing for tailored therapy, suggesting sequential daratumumab and tafamidis as a viable strategy for this complex overlap. : The authors have confirmed clinical trial registration is not needed for this submission.

摘要

免疫球蛋白轻链(AL)淀粉样变性和野生型转甲状腺素蛋白(ATTRwt)淀粉样变性虽然临床表现相似,但需要不同的治疗方法。我们报告了一例罕见病例,一名75岁的心力衰竭男性被诊断为同时患有AL和ATTRwt心脏淀粉样变性。免疫组织化学和液相色谱-串联质谱法证实心脏中存在AL和ATTR淀粉样沉积物。该患者接受了基于达雷妥尤单抗的化疗,随后使用了氯苯唑酸,治疗安全有效。该病例强调了精确淀粉样蛋白分型以进行个体化治疗的必要性,提示序贯使用达雷妥尤单抗和氯苯唑酸是应对这种复杂重叠情况的可行策略。作者已确认本投稿无需临床试验注册。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fa92/12345594/7a201a410b83/JHA2-6-e70122-g001.jpg

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