Richter综合征两例报告并文献复习

 目的　通过探讨Richter 综合征（RS）的流行病学、临床病理特点、治疗和预后，提高对其生物学本质的认识。方法　报道2例RS，分析其临床特点、实验室检查、治疗过程并复习文献。结果　2例患者的临床主要表现为淋巴结肿大，活检诊断为弥漫大B细胞淋巴瘤。外周血及骨髓有大量成熟小淋巴细胞，免疫表型符合慢性淋巴细胞白血病（CLL）。采用CHOP方案化疗后1例完全缓解，另1例部分缓解。结论　RS可发生在CLL的早期，甚至在CLL诊断时就发现RS。部分患者预后不佳，及早活检具有重要临床意义。

A report of two cases with Richter's syndrome and literature review

Objective To explore the epidemiology, clinical and pathological characteristics,treatment and prognosis of Richter's syndrome (RS). Methods The clinical and laboratory feature,treatment, prognosis of two cases were reported, and the related literature was reviewed. Results The major symptom of two cases suffered with enlarged lymph nodes, and pathological examination indicated a diffuse large B cell lymphoma. A large number of mature small lymphocytes were found in peripheral blood and bone marrow, and the immune phenotype was consistent with chronic lymphocytic leukemia. CHOP regiment was used on two cases. One obtained complete remission, and the other cases partial remission. Conclusion RS may occur at early stage after CLL diagnosis. In some cases, the diagnosis of RS and CLL are concomitant.Prognosis of some patients of RS is unfavourable. It was important to take biopsy at early stage.