结内边缘带B细胞淋巴瘤伴有单克隆IgM增高两例并文献复习

Nodal marginal zone B-cell lymphoma with monoclonal IgM： 2 cases report and review of literatures

目的分析结内边缘带B细胞淋巴瘤（NMZL）伴有单克隆IgM增高病例的临床及病理特点、诊断、治疗及预后。方法对2例NMZL病例进行分析并进行文献综述。结果惰性B细胞淋巴瘤伴有单克隆免疫球蛋白升高，最常见的两种病理类型为NMZL和B—SLL／CLL。NMZL主要累及淋巴结、脾脏、骨髓，60％发病时为进展期，国际预后指数（IPI）评分为高危，诊断主要依据淋巴结病理，单核样B细胞增生及分布有重要的参考价值。Ⅰ和（或）Ⅱ期化疗疗效好，进展期疗效差。结论NMZL主要累积淋巴结、脾脏、骨髓，可伴有单克隆IgM增多，发病时多处于疾病进展期，预后不佳。

Objective To elucidate the clinical and pathological features, diagnosis, treatment and prognosis of nodal marginal zone B-cell lymphoma （NMZL）. Methods 2 patients with NMZL who has extremely elevated monoclonal IgM were analyzed and review were included. Results The common pathological diagnosis caused by the association of monoclonal gammopathy with indolent B-cell non-Hodgkin lymphomas was marginal zone lymphoma or B small cell lymphocytic lymphoma/B-cell chronic lymphocytic leukemia. According to our cases and literatures, the main distribution of involved sites in NMZL were lymph nodes, spleen and bone marrow. Sixty percent of the NMZL patients presented with advanced stage with, high risk of IPI. The diagnosis of NMZL was based on pathology of the lymph node. The proliferation and distribution of the differentiation monocytoid cell had a specified significance. Prognosis of early stage （ Ⅰ orⅡ ） NMZL were much better than that of advanced stage NMZL even the patients had received chemotherapy. Conclusion NMZL mainly involved lymph nodes, bone marrow and spleen, sporadically associated with the elevated monoclonal IgM. Most of the NMZL patients presented with advanced stage.The prognoses were poor.