Bessudo A, Kipps T J
Department of Medicine, University of California, San Diego, La Jolla 92093-0663, USA.
Leuk Lymphoma. 1995 Aug;18(5-6):367-72. doi: 10.3109/10428199509059634.
Patients with B cell chronic lymphocytic leukemia (CLL) occasionally develop high-grade B cell lymphomas that are associated with constitutional symptoms, rapidly progressive lymphadenopathy, and swift clinical deterioration. Now known as Richter syndrome, this symptom complex develops in approximately 5% of all patients with CLL. Structural and molecular analysis of the immunoglobulin (Ig) genes have allowed investigators to define the clonal relationship between the leukemia and lymphoma cells of a given patient. In most cases the aggressive lymphoma evolves from the original leukemia cell clone. However, in some cases the lymphoma apparently represents a second malignancy. Differentiation between these two types of lymphoma may have clinical significance. Further investigation is required to allow for identification of CLL patients who are at risk for developing Richter syndrome and to understand factors involved in its etiopathogenesis.
患有B细胞慢性淋巴细胞白血病（CLL）的患者偶尔会发展为高级别B细胞淋巴瘤，这些淋巴瘤与全身症状、迅速进展的淋巴结病以及快速的临床恶化相关。这种症状复合体现在被称为里氏综合征，在所有CLL患者中约有5%会出现。免疫球蛋白（Ig）基因的结构和分子分析使研究人员能够确定特定患者白血病细胞和淋巴瘤细胞之间的克隆关系。在大多数情况下，侵袭性淋巴瘤由原始白血病细胞克隆演变而来。然而，在某些情况下，淋巴瘤显然代表第二种恶性肿瘤。区分这两种类型的淋巴瘤可能具有临床意义。需要进一步研究以识别有发展为里氏综合征风险的CLL患者，并了解其发病机制中涉及的因素。
