Fredrickson T N, Lennert K, Chattopadhyay S K, Morse H C, Hartley J W
Registry of Experimental Cancers, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA.
Am J Pathol. 1999 Mar;154(3):805-12. doi: 10.1016/S0002-9440(10)65327-8.
Splenic marginal zone lymphomas (MZLs) have been found to occur at a high frequency in NFS.N mice congenic for high-expressing ecotropic murine leukemia virus (MuLV) genes from AKR and C58 mice. Based on morphological, immunological, and molecular studies of these mice, MZL is clearly recognizable as a distinct disease with a characteristic clinical behavior. MZL was staged according to the degree of accumulation and morphological change of cells within the splenic marginal zone, as follows: 1) a moderate increase in normal-looking MZ cells, judged to be prelymphomatous, and 2) MZL in three variants: i) distinct enlargement of MZ by normal-looking cells (MZL), ii) distinct enlargement of MZ by basophilic centroblast-like cells (MZL+), and iii) extensive splenic involvement by centroblast-like cells (MZL++). The rate of mitosis and apoptosis increases with lymphoma grade. In most cases, emergence of a dominant IgH clonal pattern in paired splenic biopsy and necropsy samples was correlated with progression. MZLs were transplantable and homed to the spleen. MZL may constitute a commonly occurring lymphoma type unrecognized, in part, because of the centroblastic morphology of high-grade MZL and possible overgrowth of lower-grade MZL by more aggressive follicular lymphomas.
已发现脾边缘区淋巴瘤（MZL）在携带来自AKR和C58小鼠的高表达嗜异性鼠白血病病毒（MuLV）基因的同源NFS.N小鼠中高频发生。基于对这些小鼠的形态学、免疫学和分子学研究，MZL可明确识别为一种具有特征性临床行为的独特疾病。MZL根据脾边缘区内细胞的积聚程度和形态变化进行分期，如下：1）外观正常的MZ细胞适度增加，判定为淋巴瘤前期；2）MZL有三种变体：i）外观正常的细胞使MZ明显增大（MZL），ii）嗜碱性中心母细胞样细胞使MZ明显增大（MZL+），iii）中心母细胞样细胞广泛累及脾脏（MZL++）。有丝分裂率和凋亡率随淋巴瘤分级增加。在大多数情况下，配对的脾脏活检和尸检样本中出现显性IgH克隆模式与疾病进展相关。MZL具有可移植性且归巢于脾脏。MZL可能构成一种常见的淋巴瘤类型，部分未被识别是因为高级别MZL的中心母细胞形态以及侵袭性更强的滤泡性淋巴瘤可能会使低级别MZL过度生长。
