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首页> 外文期刊>Amyloid: the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis >A case of light chain (AL) amyloidosis associated with IgD multiple myeloma (MM): clinical features, laboratory findings and outcome.
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A case of light chain (AL) amyloidosis associated with IgD multiple myeloma (MM): clinical features, laboratory findings and outcome.

机译:一例与IgD多发性骨髓瘤(MM)相关的轻链(AL)淀粉样变性病:临床特征,实验室检查结果和结果。

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摘要

Quantitative measurements of circulating free immune-globulin light chains (FLC) and of biomarkers such as N-terminal pro-brain natriuretic peptide (NT-proBNP) and cardiac troponins (cTnT and cTnI) are the expression of recent diagnostic and prognostic advances in AL amyloidosis. Here, we report a patient diagnosed as having AL amyloidosis involving heart, kidney and soft tissue, in association with immunoglobulin D multiple myeloma (MM). This is an extremely rare disease, mostly associated with a poor prognosis. In our patient, the front-line therapy with thalidomide and dexamethasone was early discontinued because of toxicity, whereas the second-line therapy with melphalan and dexamethasone produced complete hematological response and improvement in the involved organs, still maintained after a 3.10 year follow-up. Circulating FLC, NT-ProBNP and cTnI were measured in the context of diagnostic and monitoring assessments, demonstrating a significant progressive reduction of lambda FLC and NT-ProBNP that could be related to the treatment response and long survival.
机译:循环中的游离免疫球蛋白轻链(FLC)和N端脑钠肽(NT-proBNP)和心肌肌钙蛋白(cTnT和cTnI)等生物标志物的定量测量是AL近期诊断和预后进展的表达淀粉样变性。在这里,我们报告了一名被诊断患有AL淀粉样变性的患者,包括心脏,肾脏和软组织,并伴有免疫球蛋白D多发性骨髓瘤(MM)。这是一种极为罕见的疾病,主要与预后不良有关。在我们的患者中,由于毒性,早期停用了沙利度胺和地塞米松一线治疗,而美法仑和地塞米松二线治疗对受累器官产生了完全的血液学反应和改善,在随访3.10年后仍保持不变。在诊断和监测评估的背景下对循环中的FLC,NT-ProBNP和cTnI进行了测量,证明了λFLC和NT-ProBNP的显着进行性减少可能与治疗反应和长期生存有关。

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