Mollejo Manuela, Camacho Francisca I, Algara Patricia, Ruiz-Ballesteros Elena, García Juan F, Piris Miguel A
Department of Genetics and Pathology, Hospital Virgen de la Salud, Toledo, Spain.
Hematol Oncol. 2005 Sep-Dec;23(3-4):108-18. doi: 10.1002/hon.762.
Splenic marginal zone lymphoma (SMZL) and nodal marginal zone lymphoma (NMZL) are newly defined, separate clinicopathological entities. Both are rare lymphoma types, with low reproducibility in the diagnosis, although a conjunction of molecular and clinical studies seems to be now facilitating a more accurate diagnosis and understanding of the neoplastic process. SMZL is a disease involving the spleen, bone marrow and peripheral blood since the initial manifestations of the disease. The diagnosis has been until very recently based on the pathological study of the spleen with the conjunction of the clinical features, although the integration of the morphology in bone marrow and peripheral blood with the immunophenotype and molecular characteristics of the tumour makes a more accurate diagnosis now possible. The most frequent molecular alteration found in SMZL is allelic loss at the 7q chromosomal region. SMZL is an indolent lymphoma, although there is small subset of patients in which it follows an aggressive course. Molecular studies of SMZL are starting to reveal new diagnostic and prognostic markers, and to identify new potentially useful therapeutic targets. Nodal marginal zone lymphoma is a B-cell neoplasm originated in the lymph node, whose histology resembles the nodal infiltration by MALT- or Splenic-type marginal zone lymphoma, in the absence of clinical evidence of extranodal or spleen disease. The lack of characteristic phenotypic or molecular diagnostic findings is still hampering the reproducibility of this diagnosis. Here we review the main morphological and immunophenotypical markers, discussing the differential with other overlapping entities, singularly follicular lymphoma. Specific therapeutic protocols and prognostic factors are required to more precisely define this tumour.
脾边缘区淋巴瘤（SMZL）和结内边缘区淋巴瘤（NMZL）是新定义的、独立的临床病理实体。二者均为罕见的淋巴瘤类型，诊断的可重复性较低，不过分子研究与临床研究相结合似乎正在促进对肿瘤过程更准确的诊断和理解。SMZL是一种从疾病初始表现起就累及脾脏、骨髓和外周血的疾病。直到最近，其诊断一直基于脾脏病理研究并结合临床特征，尽管将骨髓和外周血中的形态学与肿瘤的免疫表型及分子特征相结合，现在已使更准确的诊断成为可能。在SMZL中发现的最常见分子改变是7号染色体区域的等位基因缺失。SMZL是一种惰性淋巴瘤，不过有一小部分患者病程呈侵袭性。对SMZL的分子研究开始揭示新的诊断和预后标志物，并识别新的潜在有用治疗靶点。结内边缘区淋巴瘤是一种起源于淋巴结的B细胞肿瘤，其组织学类似于MALT型或脾型边缘区淋巴瘤的结内浸润，且无结外或脾脏疾病的临床证据。缺乏特征性的表型或分子诊断结果仍妨碍该诊断的可重复性。在此，我们综述主要的形态学和免疫表型标志物，讨论与其他重叠实体（尤其是滤泡性淋巴瘤）的鉴别。需要特定的治疗方案和预后因素来更精确地界定这种肿瘤。
