Richter综合征的诊断与治疗研究进展

Richter综合征(RS)是一种由低度恶性淋巴细胞增殖性疾病,向更高度恶性淋巴细胞增殖性疾病转化和(或)并发的一种疾病,多为B细胞疾病之间的转化.导致RS转化的影响因素复杂,发病率低,现行治疗方案对该病患者的预后差,诊断金标准为活组织检查.目前,RS主要治疗方案包括化疗、造血干细胞移植(HSCT)及新药试验等,部分年轻患者可从HSCT治疗中获益.若在RS转化早期及时诊断,选择合适治疗方案,则可能延长患者生存期.笔者拟就目前对RS的发病机制及其诊断与治疗的相关研究进展进行综述.

Research progress of diagnosis and treatment of Richter syndrome

Richter syndrome (RS) is defined as the transformation of a low-grade lymphoproliferative disorder into a more aggressive lymphoproliferative disorder,which mainly transform between B-cell diseases.Its conversion factors are complicated.RS has low incidence and poor prognosis.Tissue biopsy is considered as the gold standard for the diagnosis of RS.At present,the main treatment strategies are chemotherapy,hematopoietic stem cell transplantation (HSCT),taking part in a clinical trial of a new medicine,and so on.Some young patients may benefit from HSCT.The discovery of early transformation and selecting the appropriate treatment can be used to prolong patients' survival.The review is proposed to review the current pathogenesis and related research progress of the diagnosis and treatment of RS.