Lane P K, Townsend R M, Beckstead J H, Corash L
Department of Laboratory Medicine, University of California, San Francisco 94143.
Am J Clin Pathol. 1988 Feb;89(2):254-9. doi: 10.1093/ajcp/89.2.254.
Central nervous system (CNS) involvement in Richter's syndrome has not been previously described. This report describes a 45-year-old man with the simultaneous occurrence of B-cell chronic lymphocytic leukemia (CLL), extramedullary large cell non-Hodgkin's lymphoma (NHL), and malignant lymphoid meningeal involvement. In this case, peripheral blood lymphocytes, cerebrospinal fluid (CSF) lymphoblasts, and malignant cells in surgical biopsy tissue obtained from a soft tissue mass all stained concordantly for immunoglobulin isotypes and for B-cell immunophenotypic markers, supporting the hypothesis of a clonal origin for the three malignant cell populations. These observations suggest that the different tumors in Richter's syndrome (CLL and NHL) may represent the clonal progression of a common neoplasm rather than independent neoplastic events. Richter's syndrome and other transformations of lymphoid malignancies (prolymphocytic transformation of CLL, blast crisis of CLL, and blastic transformation of NHL) may all represent possible routes of progression in the natural history of a single neoplasm. The present case also suggests that, in patients with B-cell CLL with CNS symptoms, the possibility of blastic transformation presenting as CNS lymphoma deserves consideration.
中枢神经系统（CNS）受累于里氏综合征此前尚未见报道。本报告描述了一名45岁男性，同时发生B细胞慢性淋巴细胞白血病（CLL）、髓外大细胞非霍奇金淋巴瘤（NHL）和恶性淋巴细胞性脑膜受累。在该病例中，外周血淋巴细胞、脑脊液（CSF）淋巴母细胞以及从软组织肿块获取的手术活检组织中的恶性细胞，对免疫球蛋白同种型和B细胞免疫表型标志物的染色均一致，支持这三种恶性细胞群起源于克隆的假说。这些观察结果表明，里氏综合征中的不同肿瘤（CLL和NHL）可能代表一种常见肿瘤的克隆进展，而非独立的肿瘤事件。里氏综合征以及其他淋巴恶性肿瘤的转化（CLL的幼淋巴细胞转化、CLL的急变期、NHL的原始细胞转化）可能均代表单一肿瘤自然病程中的可能进展途径。本病例还提示，对于有中枢神经系统症状的B细胞CLL患者，应考虑原始细胞转化表现为中枢神经系统淋巴瘤的可能性。
