Richter综合征(Richter syndro me,RS)指在慢性淋巴细胞白血病(chronic lymphocytic leukemia,CLL)或小淋巴细胞淋巴瘤(small lymphocytic lymphoma,SLL)的背景下,疾病向侵袭性淋巴瘤进展转化的临床过程.RS转化是恶性血液病进展的一种临床表现,患者在出现RS转变时提示预后差.目前,全球范围内还未形成统一的RS治疗标准.本文回顾性分析2例从CLL向弥漫大B细胞淋巴瘤(diffuse large B-cell lymphoma,DLBCL)转化的RS患者的治疗方案及预后,探讨化疗后采用造血干细胞移植或嵌合抗原受体T细胞(chimeric antigen receptor T cell,CAR-T)免疫治疗等策略治疗RS的可能性,以为临床实践提供参考与借鉴.
Diffuse large B-cell type Richter syndrome in 2 cases and literature review
Richter syndrome(RS)refers to the clinical process of disease progression and transformation to aggressive lymphoma on the basis of chronic lymphocytic leukemia(CLL)or small lymphocytic lymphoma(SLL).RS transformation is a clinical manifestation of the progression of malignant hematological diseases,and it suggests a poor prognosis when the patient has RS transformation.Currently,there is no uniform standard for RS treatment worldwide.In this paper,the treatment regimens and prognosis of two cases with RS who transformed from CLL to diffuse large B-cell lymphoma(DLBCL)are retrospectively analyzed,and the possibility of treating RS with hematopoietic stem cell transplantation or chimeric antigen receptor T cell(CAR-T)immunotherapy after chemotherapy is discussed,in order to provide a reference for the clinical practice.
Chronic lymphocytic leukemiaDiffuse large B-cell lymphomaRichter syndromeStem cell transplantationChimeric antigen receptor T cellImmunotherapyClinical diagnosis
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