Lee A, Skelly M E, Kingma D W, Medeiros L J
Department of Pathology, Rhode Island Hospital, Providence 02903.
Am J Clin Pathol. 1995 Mar;103(3):348-52. doi: 10.1093/ajcp/103.3.348.
A 70-year-old woman with a 2-year history of B-cell chronic lymphocytic leukemia (CLL) developed headache, fever, chills, and weakness. Bone marrow examination revealed both CLL and large cell immunoblastic lymphoma (Richter's syndrome). As expected, the CLL was of B-cell lineage. The neoplasm expressed low-density monotypic IgM lambda, the pan-B-cell antigens CD19, CD20, and CDw75, and the CD5 and CD43 antigens. The large cell immunoblastic lymphoma was of T-cell lineage, positive for the CD45RB, CD3, CD45RO, and CD43 antigens, and negative for the CD20 and CDw75 antigens. Both neoplastic components were negative for Epstein-Barr virus RNA and latent membrane protein. Although 3% to 5% of patients with B-cell CLL may develop higher-grade lymphoma, usually the lymphoma is of B-cell lineage and often represents a histologic manifestation of clonal evolution. Less commonly, B-CLL patients may develop transformation to a higher grade tumor that resembles Hodgkin's disease. Both the usual form of Richter's syndrome and particularly the Hodgkin's variant of Richter's syndrome may be associated with Epstein-Barr virus. Patients with B-cell CLL rarely develop a higher grade lymphoma of T-cell lineage. To our knowledge, only one other example has been reported in the literature. Epstein-Barr virus was not associated with either neoplasm in this case.
一名患有B细胞慢性淋巴细胞白血病（CLL）2年的70岁女性出现头痛、发热、寒战和虚弱症状。骨髓检查发现同时存在CLL和大细胞免疫母细胞性淋巴瘤（里氏综合征）。正如预期的那样，CLL属于B细胞谱系。该肿瘤表达低密度单型IgMλ、泛B细胞抗原CD19、CD20和CDw75，以及CD5和CD43抗原。大细胞免疫母细胞性淋巴瘤属于T细胞谱系，对CD45RB、CD3、CD45RO和CD43抗原呈阳性，对CD20和CDw75抗原呈阴性。两种肿瘤成分的爱泼斯坦-巴尔病毒RNA和潜伏膜蛋白均为阴性。虽然3%至5%的B细胞CLL患者可能会发展为高级别淋巴瘤，但通常该淋巴瘤属于B细胞谱系，且往往代表克隆进化的组织学表现。较少见的是，B-CLL患者可能会转化为类似霍奇金病的高级别肿瘤。里氏综合征的常见形式，尤其是里氏综合征的霍奇金变异型，可能与爱泼斯坦-巴尔病毒有关。B细胞CLL患者很少发展为T细胞谱系的高级别淋巴瘤。据我们所知，文献中仅报道过另外一例。在该病例中，两种肿瘤均与爱泼斯坦-巴尔病毒无关。
