Harbhajanka Aparna, Brickman Arlen, Park Ji-Weon, Reddy Vijaya B, Bitterman Pincas, Gattuso Paolo
Department of Pathology, Rush University Medical Center, Chicago, Illinois.
Diagn Cytopathol. 2016 Sep;44(9):742-7. doi: 10.1002/dc.23523. Epub 2016 Jul 19.
Myelomatous effusions (ME) of the serous cavities are rare. Identification of the atypical plasma cells in the body fluids may be critical for prognostic considerations.
We retrospectively reviewed clinicopathologic findings of 21 serous effusion specimens from 13 cases of ME including 10 pleural, two concurrent pleural and pericardial, and one peritoneal from 1994 to 2014.
All 13 patients had bone marrow biopsy-proven plasma cell myeloma (PCM), including one plasmablastic-variant, one anaplastic-variant and one plasma cell leukemia. The time between the bone marrow diagnoses to serous cavity involvement ranged from 43 days to 9 years (mean 2.6 years, median 1.8 years). Monoclonal protein types showed predominant IgA subtypes (50%) including IgA-kappa (2), IgA-lambda (3), IgG-kappa (2), IgG-lambda (1) free kappa chain (1) and free lambda chain (1) and three unknown. All 13 patients died of disease (median survival 32 days). Concurrent imaging studies showed evidence of adjacent local disease. Cytology of the serous fluids revealed abundant plasma cells with varying degrees of atypia, including large cells with increased N/Cratios, coarse chromatin, and prominent nucleoli. All were CD138 positive. Of these, Cytology confirmed malignancy in 19/21(90%) cases. Five cases were positive for kappa-light chain and eight cases for lambda-light chain. Cytogenetics of 3/7 cases showed normal karyotype and 4/7 cases showed complex-karyotype. The patients with kappa light-chain had better survival compared to lambda light-chain (p = 0.051, log rank test).
ME in PCM can present early in the natural history of the disease or very late. The preponderance of IgA-myeloma and complex-cytogenetics abnormalities is a noteworthy finding. Although there are different hypotheses regarding how ME develops in PCM, our finding supports the hypothesis that direct spread of PCM into serous cavities is a likely mechanism. Diagn. Cytopathol. 2016;44:742-747. © 2016 Wiley Periodicals, Inc.
浆膜腔骨髓瘤性积液(ME)较为罕见。体液中不典型浆细胞的识别对于预后考量可能至关重要。
我们回顾性分析了1994年至2014年间13例ME患者的21份浆膜腔积液标本的临床病理特征,其中包括10例胸膜积液、2例同时合并胸膜和心包积液以及1例腹膜积液。
所有13例患者均经骨髓活检证实为浆细胞骨髓瘤(PCM),包括1例浆母细胞变异型、1例间变性变异型和1例浆细胞白血病。从骨髓诊断到浆膜腔受累的时间为43天至9年(平均2.6年,中位数1.8年)。单克隆蛋白类型以IgA亚型为主(50%),包括IgA-κ(2例)、IgA-λ(3例)、IgG-κ(2例)、IgG-λ(1例)、游离κ链(1例)和游离λ链(1例),另有3例未知。所有13例患者均死于疾病(中位生存期32天)。同时进行的影像学检查显示有邻近局部病变的证据。浆膜腔积液细胞学检查发现大量具有不同程度异型性的浆细胞,包括核质比增加、染色质粗糙和核仁突出的大细胞。所有细胞均CD138阳性。其中,细胞学检查确诊19/21(90%)例为恶性。5例κ轻链阳性,8例λ轻链阳性。3/7例细胞遗传学检查显示核型正常,4/7例显示复杂核型。κ轻链患者的生存期优于λ轻链患者(p = 0.051,对数秩检验)。
PCM中的ME可在疾病自然史的早期或极晚期出现。IgA骨髓瘤和复杂细胞遗传学异常占优势是一个值得关注的发现。尽管关于PCM中ME的发生有不同假说,但我们的发现支持PCM直接播散至浆膜腔是一种可能机制的假说。诊断细胞病理学。2016;44:742 - 747。©2016威利期刊公司。