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目的:提高对脾边缘区淋巴瘤(splenic marginal zone lymphoma,SMZL)的认识。方法:详细报告1例典型患者临床及实验室特征,并复习相关文献。结果:SMZL是一少见的原发于脾脏的低度恶性B细胞淋巴瘤。临床以脾脏明显肿大、淋巴细胞增多为特征,易浸润骨髓,可合并自身免疫疾患。肿瘤细胞表达成熟B细胞免疫表型,CD-5、CD-10、CD-23、CD-103,不表达T细胞相关分化抗原。结论:SMZL起病潜隐,进展缓慢,生存期长,容易漏诊。糖皮质激素和环孢菌素A治疗SMZL合并自身免疫性溶血性贫血(AIHA)近期疗效好,对SMZL本身也有一定治疗作用。
Objective: To improve the understanding of splenic marginal zone lymphoma (SMZL). Methods: The clinical and laboratory characteristics of a typical patient were reported in detail and relevant literature was reviewed. Results: SMZL is a rare low-grade primary malignant B-cell lymphoma of the spleen. Clinical obvious spleen enlargement, characterized by lymphocytosis, easily infiltrated bone marrow, may be associated with autoimmune disorders. Tumor cells expressed mature B cell immunophenotypes, CD-5, CD-10, CD-23, CD-103, and did not express T cell associated differentiation antigens. Conclusion: The onset of SMZL is latent, with slow progress, long survival period and easy missed diagnosis. Corticosteroids and cyclosporin A SMZL combined with autoimmune hemolytic anemia (AIHA) short-term curative effect, SMZL itself has some therapeutic effect.