原发性淀粉样变性伴免疫球蛋白A型多发性骨髓瘤1例报告

Nuri Karadurmus , İsmail Erturk , Mustafa Çakar , Battal Altun , Kenan Saglam , Fikret Arpaci
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引用次数: 0

摘要

原发性(AL型)淀粉样变性是最常见的系统性淀粉样变性。发病的原因是免疫球蛋白轻链(LC)原纤维在肾、心、肠等器官的细胞外沉积。原发性淀粉样变性和多发性骨髓瘤均涉及克隆浆细胞增殖。独特的血液学和生化实验室结果可能有助于早期诊断。在这里,我们提出一个60岁的男性,有一个特殊的临床过程,Ig a型多发性骨髓瘤伴全身性淀粉样变性,引起肾病综合征,完全性肠结肠炎和吸收不良。我们对这种罕见且经常致命的疾病的全面概述旨在提高对AL型淀粉样变性的认识。这可能有助于早期诊断,从而允许开始及时和特异性治疗,这对于改善疾病预后是必不可少的。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A case report: Primary amiloidosis associated with immunoglobulin A type multiple myeloma

Primary (AL type) amyloidosis is the most common form of systemic amyloidosis. The morbidity arises from extracellular deposition of immunoglobulin light chain (LC) fibrils in some organs such as the kidneys, heart and bowel. Primary amyloidosis and multiple myeloma both involve clonal plasma cell proliferation. Distinctive haematological and biochemical laboratory findings may help in early diagnosis. Here we present a 60-year-old man with an exceptional clinical course of an Ig A type multiple myeloma with generalized amyloidosis, causing nephrotic syndrome, complete intestinal colitis and malabsorbtion. Our comprehensive overview of this rare and often fatal disease aims to increase the awareness of AL type amyloidosis. This may facilitate earlier diagnosis and thus allow initiation of prompt and specific therapies, which are indispensable in order to improve disease prognosis.

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