Cioc Adina M, Jessurun José, Vercellotti Gregory M, Pambuccian Stefan E
Department of Laboratory Medicine and Pathology, University of Minnesota Medical School, Minneapolis, Minnesota.
Diagn Cytopathol. 2014 Mar;42(3):259-67. doi: 10.1002/dc.22918. Epub 2012 Sep 25.
Primary cardiac lymphomas are exceedingly rare. The presence and extent of the intracardiac mass is determined by echocardiography, computed tomography (CT), or magnetic resonance imaging (MRI); however, the diagnosis is established by endomyocardial biopsy or by pericardial or pleural effusion cytology. We describe the pleural effusion cytologic features of a primary cardiac lymphoma in a 55-year-old woman who presented with progressive shortness of breath, fatigue, mild dizziness, dull chest ache, and lower extremity edema. Transthoracic echocardiography, CT, and MRI showed a large mass centered in the right atrium and extending into the right ventricle, associated with pericardial effusion and bilateral pleural effusions. Cytologic examination of the pleural fluid showed very large pleomorphic malignant cell, some of which were binucleated and multinucleated and had anaplastic features. Flow cytometry showed a kappa monotypic population of large cells coexpressing CD5, CD19, and CD20; and immunoperoxidase stains performed on the cell block sections showed that the large neoplastic cells were positive for CD20, PAX5, CD5, and MUM1 and showed a very high proliferation rate (over 90%) by Ki67 staining. The cytologic, flow cytometry, and immunohistochemistry findings established the diagnosis of de novo CD5-positive primary cardiac diffuse large B-cell lymphoma (DLBCL), anaplastic variant, which was confirmed by the subsequent endomyocardial biopsy. This is, to the best of our knowledge, the first report of de novo CD5-positive primary cardiac diffuse large B-cell lymphoma, and the first report of the anaplastic variant of DLBCL diagnosed by effusion cytology.
原发性心脏淋巴瘤极为罕见。心脏内肿块的存在及范围通过超声心动图、计算机断层扫描（CT）或磁共振成像（MRI）来确定；然而，诊断需依靠心内膜活检或心包或胸腔积液细胞学检查来确立。我们描述了一名55岁女性原发性心脏淋巴瘤的胸腔积液细胞学特征，该患者表现为进行性气短、乏力、轻度头晕、钝性胸痛及下肢水肿。经胸超声心动图、CT和MRI显示一个以右心房为中心并延伸至右心室的巨大肿块，伴有心包积液和双侧胸腔积液。胸腔积液的细胞学检查显示有非常大的多形性恶性细胞，其中一些为双核和多核，具有间变特征。流式细胞术显示一群共表达CD5、CD19和CD20的kappa单型大细胞；对细胞块切片进行的免疫过氧化物酶染色显示，大的肿瘤细胞CD20、PAX5、CD5和MUM1呈阳性，且Ki67染色显示增殖率非常高（超过90%）。细胞学、流式细胞术和免疫组织化学检查结果确立了原发性CD5阳性原发性心脏弥漫性大B细胞淋巴瘤（DLBCL）间变亚型的诊断，随后的心内膜活检证实了这一诊断。据我们所知，这是原发性CD5阳性原发性心脏弥漫性大B细胞淋巴瘤的首例报告，也是通过积液细胞学诊断的DLBCL间变亚型的首例报告。
