Gualco Gabriela, Chioato Lucimara, Van Den Berg Anke, Weiss Lawrence M, Bacchi Carlos E
Consultoria em Patologia, Botucatu, São Paulo, Brazil.
Appl Immunohistochem Mol Morphol. 2009 Jan;17(1):72-6. doi: 10.1097/pai.0b013e31817c551f.
Composite lymphomas are rare and defined as hematopoietic neoplasms with more than 1 malignant lymphomatous clone showing different phenotypic features. Of all possible combinations between non-Hodgkin lymphomas, B cell or T cell, and Hodgkin lymphoma, the least frequent are the ones combining T-cell non-Hodgkin lymphoma and classic Hodgkin lymphoma. We report a case of a 55-year-old woman with cervical and mediastinal lymphadenopathy, fever, weight loss, and night sweats. A cervical lymph node biopsy revealed a composite lymphoma with classic Hodgkin lymphoma and peripheral T-cell lymphoma components. The bone marrow was not involved. The patient refused treatment and died of disease progression 2 months after diagnosis. The biopsied lymph node showed 2 distinct populations, one composed of large cells including typical Reed-Sternberg cells and their variants, with expression of CD30, CD15, PAX5, and LMP-1. The other component was more abundant and comprised polymorphic medium-sized cells with convoluted nuclei; CD3, CD5, CD2, and CD4 expression; and negativity for CD30, cytotoxic granules, and B-cell markers. Epstein-Barr virus DNA of subtype A was identified only in the Hodgkin cells. Clonal T-cell receptor gamma and beta gene rearrangements were detected in the T-cell component, whereas monoclonal immunoglobulin H gene rearrangement was found in the Hodgkin cells.
复合淋巴瘤较为罕见，被定义为具有超过1个显示不同表型特征的恶性淋巴瘤克隆的造血系统肿瘤。在所有非霍奇金淋巴瘤（B细胞或T细胞）与霍奇金淋巴瘤之间可能的组合中，最少见的是合并T细胞非霍奇金淋巴瘤和经典霍奇金淋巴瘤的情况。我们报告1例55岁女性患者，有颈部和纵隔淋巴结肿大、发热、体重减轻及盗汗。颈部淋巴结活检显示为具有经典霍奇金淋巴瘤和外周T细胞淋巴瘤成分的复合淋巴瘤。骨髓未受累。患者拒绝治疗，诊断后2个月因疾病进展死亡。活检的淋巴结显示出2个不同的细胞群，一个由大细胞组成，包括典型的里德-斯腾伯格细胞及其变异型，表达CD30、CD15、PAX5和LMP-1。另一个成分更为丰富，由核呈卷曲状的多形性中等大小细胞组成；表达CD3、CD5、CD2和CD4；对CD30、细胞毒性颗粒和B细胞标志物呈阴性。仅在霍奇金细胞中鉴定出A型爱泼斯坦-巴尔病毒DNA。在T细胞成分中检测到克隆性T细胞受体γ和β基因重排，而在霍奇金细胞中发现单克隆免疫球蛋白H基因重排。
