Weisenburger D D
Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha 68198.
Leukemia. 1991;5 Suppl 1:26-9.
Recent immunopathologic studies have demonstrated that primary follicles and the mantle zones of secondary follicles are composed largely of virgin B lymphocytes which migrate from the bone marrow to these areas, and are the precursor cells of germinal centers. Non-Hodgkin's lymphomas corresponding to these immature B cells include mantle zone lymphoma (MZL), a primary follicle variant of MZL without reactive germinal centers, and diffuse intermediate lymphocytic (centrocytic) lymphoma. Diffuse intermediate lymphocytic lymphoma (DILL) is considered a late stage in the progression of MZL. Cytologically, these lymphomas usually resemble their normal cellular counterparts and consist predominantly of atypical small lymphoid cells with slightly-irregular and indented nuclei, moderately-coarse chromatin, inconspicuous nucleoli, and scant cytoplasm. Small lymphocytic, cerebriform and blastic variants have also been described. In smears and touch preparations, the neoplastic cells are usually prolymphocytes. Immunologically, the cells have features of virgin B cells, bearing pan-B cell antigens along with monoclonal surface IgM, with or without surface IgD, and CD5 (Leu 1) antigen, and lacking common acute lymphocytic leukemia associated (CALLA) antigen. Cytogenetically, the t(11;14)(q13;q32) has been associated with this group of lymphomas, and expression of the putative cellular oncogene bcl-1 (11q13) has been demonstrated in 30-50% of cases. Clinically, the patients have a median age of 60 years and usually present with advanced stage disease. Splenomegaly, often massive, is present in 80% of those with MZL. Patients with MZL have a significantly longer median survival (74-77 months) than those with DILL (30-33 months), and survival in both groups is significantly prolonged if a complete clinical remission is attained. Based on clinical studies, MZL should be considered a low grade lymphoma and DILL should be considered an intermediate grade lymphoma by Working Formulation criteria. The lymphomas of primary follicle/mantle zone origin are a distinct clinicopathologic entity biologically analogous to the follicular and diffuse lymphomas of germinal center origin, from which they should be distinguished in current and future classifications of non-Hodgkin's lymphoma.
近期的免疫病理学研究表明，初级滤泡和次级滤泡的套区主要由从骨髓迁移至这些区域的处女B淋巴细胞组成，它们是生发中心的前体细胞。与这些未成熟B细胞相对应的非霍奇金淋巴瘤包括套区淋巴瘤（MZL）、无反应性生发中心的MZL初级滤泡变异型以及弥漫性中等淋巴细胞（中心细胞）淋巴瘤。弥漫性中等淋巴细胞淋巴瘤（DILL）被认为是MZL进展的晚期阶段。在细胞学上，这些淋巴瘤通常与其正常细胞对应物相似，主要由非典型小淋巴细胞组成，细胞核略不规则且有凹陷，染色质中等粗糙，核仁不明显，细胞质稀少。也描述了小淋巴细胞、脑回状和母细胞样变异型。在涂片和印片标本中，肿瘤细胞通常是幼淋巴细胞。在免疫学上，这些细胞具有处女B细胞的特征，带有全B细胞抗原以及单克隆表面IgM，有或无表面IgD，以及CD5（Leu 1）抗原，并且缺乏常见的急性淋巴细胞白血病相关（CALLA）抗原。在细胞遗传学上，t(11;14)(q13;q32)与这组淋巴瘤相关，并且在30% - 50%的病例中已证实假定的细胞癌基因bcl - 1（11q13）的表达。临床上，患者的中位年龄为60岁，通常表现为晚期疾病。80%的MZL患者存在脾肿大，通常较为显著。MZL患者的中位生存期（74 - 77个月）明显长于DILL患者（30 - 33个月），并且如果达到完全临床缓解，两组患者的生存期都会显著延长。根据临床研究，按照工作分类标准，MZL应被视为低度淋巴瘤，DILL应被视为中度淋巴瘤。起源于初级滤泡/套区的淋巴瘤是一种独特的临床病理实体，在生物学上类似于起源于生发中心的滤泡性和弥漫性淋巴瘤，在当前和未来的非霍奇金淋巴瘤分类中应将它们区分开来。
