Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA.
Hum Pathol. 2010 Apr;41(4):540-51. doi: 10.1016/j.humpath.2009.09.007. Epub 2009 Dec 11.
Classic splenic marginal zone lymphomas are CD5-, CD10-, CD23-, CD43-, and usually IgD+ with biphasic white pulp nodules. However, the 2008 World Health Organization classification accepts splenic marginal zone lymphomas with monophasic marginal zone-like white pulp nodules and recognizes a group of unclassifiable splenic small B-cell lymphomas. To explore the relationship of classic splenic marginal zone lymphomas to these other less well-defined splenic lymphomas, a multiparameter study of 47 splenic marginal zone lymphomas and unclassifiable splenic small B-cell lymphomas was performed. Seventeen of 31 splenic marginal zone lymphomas were biphasic, and 14 were monophasic (90%-100% marginal zone-like white pulp nodules). Sixteen cases were unclassifiable splenic small B-cell lymphomas, most lacking a marginal zone-type component. There were many clinical similarities between the 3 groups, including similar survivals. Monophasic and unclassifiable cases were less likely to have a typical splenic marginal zone lymphoma phenotype (28.6%, 23.1%) compared with biphasic cases (86.7%), usually because of IgD negativity (P < .003). Thirty-four of 42 (81%) cases had cytogenetic abnormalities by fluorescence in situ hybridization; and 17 of 20 (85%), by classical cytogenetics. The most frequent fluorescence in situ hybridization abnormalities among the splenic marginal zone lymphomas were del(7)(q31) (26%), +12 (25%), and +3q27 (27%); and among the unclassifiable cases, +12 (50%) and +3q27 (36%). Five of 6 unclassifiable cases with exclusively small non-marginal zone-like lymphocytes involving both white and red pulp had +12 compared with 9 of 34 other cases (P < .02). CDK6 (2 cases) and BCL3 (1 case) rearrangements were only seen in the unclassifiable group. These results support including both biphasic and monophasic cases as splenic marginal zone lymphomas, but suggest that the lack of a non-marginal zone-like population in the monophasic group is associated with some biologic differences. They also demonstrate a relatively large proportion of unclassifiable cases, including a group with frequent +12.
经典脾边缘区淋巴瘤为 CD5-、CD10-、CD23-、CD43-，通常为 IgD+，伴有双相白髓小结节。然而，2008 年世界卫生组织分类接受了具有单相边缘区样白髓小结节的脾边缘区淋巴瘤，并承认了一组无法分类的脾小 B 细胞淋巴瘤。为了探讨经典脾边缘区淋巴瘤与这些其他定义不明确的脾淋巴瘤的关系，对 47 例脾边缘区淋巴瘤和无法分类的脾小 B 细胞淋巴瘤进行了多参数研究。31 例脾边缘区淋巴瘤中有 17 例为双相，14 例为单相（90%-100%边缘区样白髓小结节）。16 例为无法分类的脾小 B 细胞淋巴瘤，大多数缺乏边缘区型成分。这 3 组之间存在许多临床相似之处，包括相似的存活率。与双相病例（86.7%）相比，单相和无法分类的病例更不可能具有典型的脾边缘区淋巴瘤表型（28.6%、23.1%），通常是因为 IgD 阴性（P<.003）。通过荧光原位杂交，42 例中的 34 例（81%）存在细胞遗传学异常；通过经典细胞遗传学，20 例中的 17 例（85%）存在细胞遗传学异常。脾边缘区淋巴瘤中最常见的荧光原位杂交异常为 del(7)(q31)（26%）、+12（25%）和+3q27（27%）；无法分类的病例中，+12（50%）和+3q27（36%）最为常见。6 例仅累及白髓和红髓的小非边缘区样淋巴细胞的无法分类病例中，有 5 例存在+12，而 34 例其他病例中仅有 9 例存在+12（P<.02）。CDK6（2 例）和 BCL3（1 例）重排仅见于无法分类的病例组。这些结果支持将双相和单相病例均归入脾边缘区淋巴瘤，但提示单相组中缺乏非边缘区样细胞群与某些生物学差异有关。它们还表明无法分类的病例比例相对较大，包括一组经常出现+12 的病例。
