Department of Pathology and Laboratory Medicine, University of Miami and Jackson Health System, Miami, Florida.
Department of Pathology and Laboratory Medicine, Rhode Island Hospital and Lifespan Medical Center, The Warren Alpert Medical School of Brown University, Providence, Rhode Island.
R I Med J (2013). 2022 Aug 1;105(6):41-45.
Plasma cell myeloma (PCM) is defined as a clonal disease of terminally differentiated plasma cells that secrete immunoglobulin. The biologic underpinnings of IgA-type multiple myeloma's (IgAMM) aggressive nature, including its increased morbidity and mortality, have not been elucidated. We describe the clinical, phenotypic, and cytogenetic characteristics of IgA-MM. Flow-cytometry analysis was performed to phenotype clonal plasma cell populations, and interface with fluorescent in situ hybridization (iFISH) to exploit cytogenetics to determine risk stratification; 68.1% of cases were of intermediate or high risk. On flow cytometry, samples from our IgA-PCM cohort revealed less frequent CD56 expression when compared to samples with other PCM subtypes. Our study demonstrated lower frequency of CD56 expression (52.8%). We hypothesize that loss of CD56 may play a significant role in the aggressive behavior of IgA-PCM due to the loss of cell-to-cell adhesion resulting in a higher propensity for extramedullary presentation.
浆细胞骨髓瘤(PCM)被定义为终末分化浆细胞的克隆性疾病,其分泌免疫球蛋白。尚未阐明 IgA 型多发性骨髓瘤(IgAMM)侵袭性的生物学基础,包括其增加的发病率和死亡率。我们描述了 IgA-MM 的临床、表型和细胞遗传学特征。进行流式细胞术分析以表型克隆性浆细胞群体,并与荧光原位杂交(iFISH)接口利用细胞遗传学确定风险分层;68.1%的病例为中危或高危。在流式细胞术上,与其他 PCM 亚型相比,我们的 IgA-PCM 队列的样本中 CD56 的表达频率较低。我们的研究显示 CD56 的表达频率较低(52.8%)。我们假设由于细胞间粘附的丧失导致骨髓外表现的倾向性增加,CD56 的丧失可能在 IgA-PCM 的侵袭性行为中起重要作用。