Arcaini Luca, Paulli Marco, Boveri Emanuela, Magrini Umberto, Lazzarino Mario
Division of Hematology, IRCCS Policlinico San Matteo, University of Pavia, Italy.
Haematologica. 2003 Jan;88(1):80-93.
The marginal zone is an anatomically distinct B-cell compartment of lymphoid tissue with an abundant antigenic influx. Among marginal zone-derived lymphomas the WHO classification listed, in addition to extranodal marginal zone B-cell lymphoma of MALT type, two other marginal zone B-cell neoplasms: splenic marginal zone B-cell lymphoma (+/- villous lymphocytes) and nodal marginal zone B-cell lymphoma (+/- monocytoid B cells). These two entities are well characterized histologically, but specific biological markers are lacking. Treatment options are heterogeneous, including a watch-and-wait policy, surgery with or without chemotherapy, purine analogs, and interferon. No prospective studies have been conducted so far.
Clinical and pathologic data were reviewed by searches of the published medical literature, including searches in PubMed , important printed publications, and abstracts presented at recent hematology and pathology meetings.
Splenic and nodal marginal zone lymphomas are typical low-grade lymphomas with an indolent course. A subset of patients, however, presents with more aggressive disease and have a shorter survival. Clinical and biological prognostic factors identified in reported series are heterogeneous. The role played by hepatitis C virus (HCV) in marginal zone lymphomas is not fully elucidated, but there is demonstration that eradication of HCV infection in splenic lymphoma with villous lymphocytes causes regression of the lymphoma. The optimal treatment has not yet been identified. Retrospective series, however, show that splenectomy is a good option if symptoms from the presence of spleen enlargement or cytopenias need to be treated. The utility of purine analogs and of anti-CD20 immunotherapy needs to be clarified in prospective trials.
Clinicians and pathologists should co-operate to define stringent diagnostic criteria for these indolent disorders. The optimal therapeutic approach and the role of new treatments need to be assessed in prospective clinical trials.
边缘区是淋巴组织中解剖学上独特的B细胞区室，有丰富的抗原流入。在源自边缘区的淋巴瘤中，世界卫生组织分类除了列出黏膜相关淋巴组织型结外边缘区B细胞淋巴瘤外，还有另外两种边缘区B细胞肿瘤：脾边缘区B细胞淋巴瘤（±绒毛状淋巴细胞）和淋巴结边缘区B细胞淋巴瘤（±单核细胞样B细胞）。这两种实体在组织学上有很好的特征描述，但缺乏特异性生物学标志物。治疗选择多种多样，包括观察等待策略、手术（有无化疗）、嘌呤类似物和干扰素。目前尚未进行前瞻性研究。
通过检索已发表的医学文献来回顾临床和病理数据，包括在PubMed、重要印刷出版物以及近期血液学和病理学会议上发表的摘要中进行检索。
脾和淋巴结边缘区淋巴瘤是典型的惰性病程低度淋巴瘤。然而，一部分患者表现为侵袭性更强的疾病，生存期较短。报道系列中确定的临床和生物学预后因素各不相同。丙型肝炎病毒（HCV）在边缘区淋巴瘤中的作用尚未完全阐明，但有证据表明，在伴有绒毛状淋巴细胞的脾淋巴瘤中根除HCV感染可使淋巴瘤消退。尚未确定最佳治疗方法。然而，回顾性系列研究表明，如果需要治疗脾肿大或血细胞减少引起的症状，脾切除术是一个不错的选择。嘌呤类似物和抗CD20免疫疗法的效用需要在前瞻性试验中加以明确。
临床医生和病理学家应合作，为这些惰性疾病确定严格的诊断标准。最佳治疗方法和新治疗方法的作用需要在前瞻性临床试验中进行评估。
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