Unusual biclonal IgA plasma cell myeloma with aberrant expression of high-risk immunophenotypes: first report of a new diagnostic and clinical challenge.

Carlos A Monroig-Rivera, Clara N Finch Cruz
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Abstract

IgA plasma cell myeloma (PCM) has been linked to molecular abnormalities that confer a higher risk for adverse patient outcomes. However, since IgA PCM only accounts for approximately 20% of all PCM, there are very few reports on high-risk IgA PCM. Moreover, no such reports are found on the more infrequent biclonal IgA PCM. Hence, we present a 65-year-old Puerto Rican female with acute abdominal pain, concomitant hypercalcemia, and acute renal failure. Protein electrophoresis with immunofixation found high IgA levels and detected a biclonal IgA gammopathy with kappa specificity. Histomorphologically, bone marrow showed numerous abnormal plasma cells (32%) replacing over 50% of the marrow stroma. Immunophenotyping analysis detected CD45-negative plasma cells aberrantly expressing CD33, CD43, OCT-2, and c-MYC. Chromosomal analysis revealed multiple abnormalities including the gain of chromosome 1q. Thus, we report on an unusual biclonal IgA PCM and the importance of timely diagnosing aggressive plasma cell neoplasms.

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异常双克隆IgA浆细胞骨髓瘤伴高危免疫表型异常表达:新诊断和临床挑战的首次报道。
IgA浆细胞骨髓瘤(PCM)与分子异常有关,导致患者不良预后的风险较高。然而,由于IgA PCM仅占所有PCM的约20%,因此关于高风险IgA PCM的报道很少。此外,在更罕见的双克隆IgA PCM中没有发现此类报道。因此,我们提出一个65岁的波多黎各女性急性腹痛,伴随高钙血症和急性肾功能衰竭。免疫固定蛋白电泳发现IgA水平高,并检测出具有kappa特异性的双克隆IgA伽玛病。骨髓组织形态学显示大量异常浆细胞(32%)取代了50%以上的骨髓基质。免疫表型分析检测到cd45阴性浆细胞异常表达CD33、CD43、OCT-2和c-MYC。染色体分析显示多种异常,包括染色体1q的增加。因此,我们报告了一个不寻常的双克隆IgA PCM和及时诊断侵袭性浆细胞肿瘤的重要性。
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来源期刊
CiteScore
5.00
自引率
4.20%
发文量
45
审稿时长
14 weeks
期刊介绍: The Journal of Pathology and Translational Medicine is an open venue for the rapid publication of major achievements in various fields of pathology, cytopathology, and biomedical and translational research. The Journal aims to share new insights into the molecular and cellular mechanisms of human diseases and to report major advances in both experimental and clinical medicine, with a particular emphasis on translational research. The investigations of human cells and tissues using high-dimensional biology techniques such as genomics and proteomics will be given a high priority. Articles on stem cell biology are also welcome. The categories of manuscript include original articles, review and perspective articles, case studies, brief case reports, and letters to the editor.
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