Cutaneous Marginal Zone Lymphomas Have Distinctive Features and Include 2 Subsets

Cutaneous marginal zone lymphomas (CMZL) were segregated in the WHO/EORTC consensus classification but grouped with other MALT lymphomas in the subsequent WHO classification. It has been suggested, however, that CMZL have distinctive features and might include 2 subsets. To address these issues, the clinicopathologic, phenotypic and, when possible, genotypic features of 29 CMZL with plasmacytic differentiation were assessed. The monotypic plasma cells had class-switched heavy chain expression in 22 cases, technically inadequate staining in 1 case (included with class-switched cases for analysis) and 6 were IgM+. The class-switched cases had a predominance of T cells in 22 out of 23 cases with a CD4:CD8>1 in 15 out of 16 cases, usually showed nodules and scattered small B cells often with IgD+ apparently nonneoplastic follicles, lacked CXCR3 B-cell expression, never showed a totally diffuse growth pattern, often had prominent mast cells, and lacked known extracutaneous involvement. The IgM+ cases showed a predominance of B cells in 5 out of 6 (P=0.0003), a diffuse proliferation of CD20+ B cells in all (P<0.0001), CXCR3+ B cells in 2 out of 5 (P<0.04), and extracutaneous disease in 3 out of 6 (P<0.008). CD21+ usually disrupted follicular dendritic meshworks were seen in 9 out of 12 class-switched and 5 out of 5 IgM+ cases. CD123+ plasmacytoid dendritic cells, PD1+ T follicular helper cells, CD25+ or FOXP3+ regulatory T cells, and TIA1+/granzyme B− cytotoxic cells were never numerous. Only 1 out of 14 tested cases showed a low-level clonal/oligoclonal T cell receptor &ggr; gene rearrangement. These findings support the presence of 2 types of cutaneous MALT lymphomas with the class-switched cases being the most distinctive but still sharing significant features with MALT lymphomas from other sites, specifically an extranodal extramedullary CD5-, CD10- indolent small B cell lymphoma with plasmacytic differentiation, frequent benign follicular structures, and not fulfilling the criteria for any other well-defined lymphoma.

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