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多发性骨髓瘤的偶然发现,表现为胸壁含浆细胞成分的淀粉样瘤。

Incidental Discovery of Multiple Myeloma, Presenting as an Amyloid Tumor with Plasmacytic Elements of the Thoracic Wall.

作者信息

Şerban Oana, Achim A, Poantă Laura Irina

出版信息

Rom J Intern Med. 2015 Jul-Sep;53(3):267-72. doi: 10.1515/rjim-2015-0035.

Abstract

Multiple myeloma is characterized by monoclonal proliferation of bone marrow plasma cells causing multiple bone lesions and overproduction of a monoclonal protein (M-protein) that could deposit in tissues (amyloidosis). Dissemination of the multiple myeloma outside the bone is rare. We present a case of a 76 years old woman presenting with dry cough. Chest X-ray showed a giant tumor of the upper right lung. Contrast enhanced CT revealed a tumor that most probably originated from the structures of the thoracic wall. The transthoracic biopsy was inconclusive. The tumor was resected and the histopathological examination showed amyloid tumor of the thoracic wall with plasmacytic elements and lambda light chains deposits. A bone marrow aspiration was performed that found diffuse plasmacytic infiltrate of 20-60% and the serum proteins electrophoresis with immunofixation revealed elevated IgA and lambda light chains. The patient was diagnosed with IgA and lambda light chains multiple myeloma with consequent AL amyloidosis presenting as thoracic mass.

摘要

多发性骨髓瘤的特征是骨髓浆细胞单克隆增殖,导致多处骨病变,并过度产生可沉积于组织的单克隆蛋白(M蛋白)(淀粉样变性)。多发性骨髓瘤在骨外播散的情况罕见。我们报告一例76岁女性,表现为干咳。胸部X线显示右上肺巨大肿瘤。增强CT显示一个很可能起源于胸壁结构的肿瘤。经胸壁活检结果不明确。肿瘤被切除,组织病理学检查显示胸壁淀粉样瘤伴浆细胞成分及λ轻链沉积。进行了骨髓穿刺,发现弥漫性浆细胞浸润达20% - 60%,血清蛋白电泳及免疫固定显示IgA和λ轻链升高。该患者被诊断为IgA和λ轻链多发性骨髓瘤,继而出现以胸壁肿块形式表现的AL淀粉样变性。

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