Objective: To investigate the clinical characteristics, response, and prognosis of splenic diffuse red pulp small B-cell lymphoma (SDRPL) . Methods: Eight cases of SDRPL were diagnosed and treated at Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, between May 2017 and April 2022. Data on the clinical features, laboratory results, bone marrow and spleen biopsy results, response, and prognosis were collected and analyzed. Results: The median age at diagnosis was 54 (42-69) years. Splenomegaly and lymphocytosis were present in all cases, and PET/CT revealed normal to slightly elevated splenic FDG uptake. All cases were in stage Ⅳ, with spleen, peripheral blood, and bone marrow but no proximal lymph nodes involved. The cytoplasm of neoplastic villous cells was abundant, and splenic pathology showed that small homogenous lymphocytes permeated the splenic sinus and splenic cord, and the white pulp atrophied. Immunohistochemistry was not typical, and B-cell markers including CD19, CD20 and CD79α were positive. After a median follow up of 35.5 (4-60) months, 7 cases were alive after splenectomy with or without chemoimmunotherapy. The patient with CCND3 P284A and MYC S146L mutation developed to B-cell prolymphocytic leukemia (B-PLL) 1 month after splenectomy and died at 16 months of follow-up. Conclusion: A rare indolent B-cell lymphoma that primarily affects the elderly, SDRPL. Most patients achieved long-term survival, but the prognosis of patients who progress to B-PLL was poor.
目的: 探究脾弥漫性红髓小B细胞淋巴瘤(SDRPL)患者的临床特征、疗效及预后。 方法: 回顾性分析2017年5月至2022年4月在上海交通大学医学院附属瑞金医院诊治的8例SDRPL患者的病历资料,对患者的临床特征、实验室检查结果、骨髓和脾脏病理、疗效与预后进行分析及总结。 结果: 8例患者中位年龄54(42~69)岁。确诊时均表现为明显的脾脏肿大,多有淋巴细胞升高,PET/CT显示脾脏代谢不高或轻度增高。临床分期均为Ⅳ期,累及脾脏、外周血和骨髓,未见外周淋巴结受累。肿瘤细胞胞质丰富,易见短毛刺状突起。脾脏病理可见形态均一的小淋巴细胞弥漫浸润脾窦及脾索,白髓萎缩。免疫表型缺乏特异性,多表达CD19、CD20、CD79α等B细胞抗原。中位随访35.5(4~60)个月,脾切除术联合或不联合免疫化疗可使7例患者获得长期生存,1例伴CCND3 P284A和MYC S146L突变的患者在脾切除术后1个月进展为B细胞幼淋巴细胞白血病(B-PLL),在随访16个月时死亡。 结论: SDRPL患者以中老年为主,临床呈惰性病程。患者大多可获得长期生存,但进展为B-PLL的患者预后较差。.
Keywords: Diagnosis; Lymphoma, B-cell; Spleen neoplasms; Therapeutics.