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为了探讨Richter综合征的临床病理特点、诊断、治疗与预后,报道1例经典型Richter综合征,通过临床表现、骨髓细胞学、淋巴结病理及免疫组化研究该病的特点,并复习有关文献。结果患者外周血及骨髓见大量成熟小淋巴细胞,细胞形态学符合慢性淋巴细胞白血病骨髓像,骨髓细胞免疫表型为CD5(+)、CD10(-)、CD19(+)、CD20(+)、CD23(+)和CD38(+);淋巴结组织病理学为弥漫性大B细胞淋巴瘤。经多周期化疗,疗效欠佳,仅生存11个月。初步研究结果提示,该例患者初诊时为慢性淋巴细胞白血病同时合并弥漫性大B细胞淋巴瘤,符合经典型Richter综合征特点,国内罕见,生存期短,预后很差。
In order to explore the clinicopathological features, diagnosis, treatment and prognosis of Richter syndrome, one case of classic Richter syndrome was reported. The clinical features, myelocytology, lymph node pathology and immunohistochemistry were used to investigate the characteristics of the disease and to review the literature. Results A large number of mature lymphocytes were found in the peripheral blood and bone marrow of patients. The morphology of the cells was in line with the bone marrow of chronic lymphocytic leukemia. The immunophenotype of bone marrow was CD5 (+), CD10 (-), CD19 (+), CD20 CD23 (+) and CD38 (+); lymph node histopathology is diffuse large B cell lymphoma. After multi-cycle chemotherapy, poor efficacy, only 11 months of survival. Preliminary results suggest that patients with newly diagnosed patients with chronic lymphocytic leukemia at the same time with diffuse large B-cell lymphoma, in line with the characteristics of classic Richter syndrome, rare in China, short survival and poor prognosis.