Richter综合征4例报道及文献复习

Clinical Reports on 4 Cases of Richter Syndrome and the Literature Review

目的分析病例并复习文献提高对Richter综合征(RS)的认识及诊疗水平。方法回顾性分析4例RS患者的临床资料并复习相关文献。结果 4例患者中有3例在治疗原发病后发生转化,1例患者出现症状时即被诊断为RS,发生转化前治疗效果尚可,但转化为RS后,治疗难度明显增大。结论 RS临床少见,转化因素复杂,化疗效果差,自体造血干细胞移植效果需要进一步观察。对于低度恶性的淋巴瘤或白血病患者,如果治疗效果欠佳,要高度警惕转化为RS的可能。

Objective To improve the level of recognition,diagnosis and treatment of Richter syndrome（RS） through analyzing cases and the literature review.Methods 4 patients of RS were studied retrospectively of clinical characteristics,and related literatures were reviewed.Results 3 patients transformed after the primary disease was treated and 1 patient was diagnosed when presenting the symptoms among the 4 patients.The treatment effect was acceptable before transforming into RS.,but after the transformation,the treatment difficulty was significantly increased.Conclusion RS is rare in clinical with complicated transforming factors and poor prognosis.The chemotherapy effect is poor and the effect of autologous hematopoietic stem cell transplantation still needs further follow-up.For low-grade lymphoma or leukemia patients,it is necessary to be aware of the possibility of transforming into RS if the treatment is ineffective.