脾边缘区淋巴瘤伴自身免疫性溶血性贫血1例并文献复习

SPLENIC MARGINAL ZONE LYMPHOMA WITH AUTOIMMUNE HEMOLYTIC ANEMIA

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 enhance the knowledge about splenic marginal zone lymphoma,SMZL. Methods: We reported the detailed clinical presentation and laboratory results of a patient with SMZL and autoimmune hemolytic anemia and reviewed the related literature. Results: SMZL, an rare entity of primary splenic B cell lymphoma, is usually a chronic disease. It manifested with marked splenomegaly,lymphocytosis and sometimes associated with autoimmune diseases.Bone marrow involvement is a common feature in SMZL. The clonal proliferative lymphocytes express positive mature B cell and negative T cell phenotype. Conclusion: SMZL is a distinct indolent disease with specific clinicobiological aspects. Treated with prednisone and cyclosporine A may led to good response for SMZL and the coexistent autoimmune hemolytic anemia.