Yamazaki Mika L, Lum Christopher A, Izumi Allan K
John A. Burns School of Medicine, University of Hawaii at Manoa, Honolulu, Hawaii, USA.
J Am Acad Dermatol. 2009 Jan;60(1):157-61. doi: 10.1016/j.jaad.2008.07.017.
The term Richter syndrome (RS) describes the transformation of chronic lymphocytic leukemia into a high-grade lymphoma. RS occurs in 3% to 10% of chronic lymphocytic leukemia cases, and its onset is often characterized by the abrupt development of systemic symptoms (eg, fever in the absence of infection, night sweats, and weight loss), progressive lymphadenopathy, and hepatosplenomegaly. RS frequently arises in the lymph nodes or bone marrow, and rarely presents with extranodal involvement, which includes the gastrointestinal tract, eye, testis, central nervous system, lung, kidney, and skin. We review the literature regarding the clinical course and treatment of RS, present a patient with primary cutaneous RS, and discuss the prognostic implications.
里氏综合征（RS）这一术语描述了慢性淋巴细胞白血病向高级别淋巴瘤的转化。RS发生于3%至10%的慢性淋巴细胞白血病病例中，其发病通常表现为全身症状（如无感染情况下的发热、盗汗和体重减轻）突然出现、进行性淋巴结病以及肝脾肿大。RS常发生于淋巴结或骨髓，很少出现结外受累，包括胃肠道、眼、睾丸、中枢神经系统、肺、肾和皮肤。我们回顾了关于RS临床病程和治疗的文献，介绍了一名原发性皮肤RS患者，并讨论了其预后意义。
