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华氏巨球蛋白血症伴血清淀粉样蛋白 A 蛋白淀粉样变性:诊断中的陷阱和基于马法兰的自体干细胞移植的成功治疗。

Waldenström's macroglobulinemia associated with serum amyloid A protein amyloidosis: pitfalls in diagnosis and successful treatment with melphalan-based autologous stem cell transplant.

机构信息

Myeloma Institute for Research and Therapy, University of Arkansas for Medical Sciences, Little Rock, Ark., USA.

出版信息

Acta Haematol. 2013;130(3):146-9. doi: 10.1159/000347036. Epub 2013 May 9.

Abstract

Waldenström's macroglobulinemia (WM) is increasingly being associated with amyloidosis particularly of the amyloid light-chain variety. We report on one of the few cases of WM associated with serum amyloid A protein (AA) amyloidosis. Autologous stem cell transplant (ASCT) is now being increasingly used for the treatment of amyloidosis, but most studies are small case series. Traditionally AA amyloid is associated with connective tissue disorders and periodic fever syndromes and has been treated by addressing the underlying condition. We present the first case of serum amyloid A being treated with melphalan-based ASCT to deal with the underlying WM and thereby control the amyloid, thus demonstrating the viability of this novel approach for the treatment of this disorder.

摘要

瓦尔登斯特伦巨球蛋白血症(WM)越来越多地与淀粉样变性,特别是轻链淀粉样变性相关。我们报告了一例罕见的 WM 伴血清淀粉样 A 蛋白(AA)淀粉样变性。自体干细胞移植(ASCT)目前越来越多地用于治疗淀粉样变性,但大多数研究都是小型病例系列。传统上,AA 淀粉样变性与结缔组织疾病和周期性发热综合征相关,并通过治疗基础疾病进行治疗。我们提出了首例使用基于美法仑的 ASCT 治疗血清淀粉样 A 的病例,以治疗基础 WM 并控制淀粉样变性,从而证明了这种治疗该疾病的新方法的可行性。

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