Tadmor Tamar, Polliack Aaron
Hematology Unit, Bnai-Zion Medical Center, Israel; The Ruth and Bruce Rappaport Faculty of Medicine, Technion, Haifa, Israel.
Department of Hematology, Hadassah University, Hospital and Hebrew University Medical School, Jerusalem, Israel.
Best Pract Res Clin Haematol. 2017 Mar-Jun;30(1-2):92-98. doi: 10.1016/j.beha.2016.08.026. Epub 2016 Nov 4.
Nodular marginal zone lymphoma (NMZL) is a small B-cell lymphoma involving only lymph nodes and is the least common form of MZL constituting about 10% of cases. Patients usually present with advanced disease which must be distinguished from extranodal MZL with lymph node spread. NMZL shares cytological and immunophenotypic features with MALT and splenic MZL, but has a less favorable prognosis than these two categories. It occurs mostly in adults and pediatric cases are rare. Different therapeutic approaches have been used in NMZL, but because of the small patient numbers involved, more definitive treatment is still anticipated. Recent studies suggest that it probably represents a separate entity within the broader indolent lymphoma category. In NMZL there is an emerging need to utilize novel agents, already available for indolent lymphomas. Prospective studies are required to evaluate their therapeutic efficacy for NMZL in the future.
结节性边缘区淋巴瘤（NMZL）是一种仅累及淋巴结的小B细胞淋巴瘤，是MZL最不常见的形式，约占病例的10%。患者通常表现为晚期疾病，必须与伴有淋巴结扩散的结外MZL相鉴别。NMZL与MALT和脾MZL具有细胞学和免疫表型特征，但预后比这两类淋巴瘤更差。它主要发生于成年人，儿科病例罕见。NMZL采用了不同的治疗方法，但由于涉及的患者数量较少，仍期待更明确的治疗。最近的研究表明，它可能代表了更广泛的惰性淋巴瘤类别中的一个独立实体。在NMZL中，越来越需要使用已用于惰性淋巴瘤的新型药物。未来需要进行前瞻性研究来评估它们对NMZL的治疗效果。
