Departmentsof Pathology, Massachusetts General Hospital, Boston, MA 02114, USA.
Am J Clin Pathol. 2010 Jan;133(1):59-69. doi: 10.1309/AJCPW64FFBTTPKFN.
Primary cutaneous marginal zone lymphoma (MZL) is a common B-cell lymphoma of skin and is characterized by an infiltrate of neoplastic marginal zone B cells typically within the marginal zones of reactive lymphoid follicles and the interfollicular region. However, in our experience, many cases have underemphasized features such as marked plasmacytic differentiation and/or a prominent T-cell component, which may obscure the neoplastic B cells and lead to misdiagnosis. We wanted to draw attention to these features and have studied 15 cases of MZL with marked plasmacytic differentiation, 10 of which had numerous T cells, some with cytologic atypia, and few B cells in the interfollicular region. Plasma cells were monotypic in all cases by in situ hybridization. By polymerase chain reaction, 6 of 8 T cell-rich cases had an IGH gene rearrangement, and none were clonal for T-cell receptor gene. We discuss the terminology, morphologic features, molecular profile, behavior, and differential diagnosis of cutaneous MZL.
原发性皮肤边缘区淋巴瘤（MZL）是一种常见的皮肤 B 细胞淋巴瘤，其特征为肿瘤性边缘区 B 细胞浸润，通常位于反应性淋巴滤泡的边缘区和滤泡间区。然而，根据我们的经验，许多病例具有被低估的特征，如明显的浆细胞分化和/或突出的 T 细胞成分，这可能会掩盖肿瘤性 B 细胞并导致误诊。我们希望引起对这些特征的关注，并研究了 15 例具有明显浆细胞分化的 MZL，其中 10 例有大量 T 细胞，其中一些具有细胞学异型性，滤泡间区的 B 细胞较少。所有病例的浆细胞通过原位杂交均呈单型性。通过聚合酶链反应，8 例 T 细胞丰富的病例中有 6 例存在 IGH 基因重排，并且没有 T 细胞受体基因的克隆性。我们讨论了皮肤 MZL 的术语、形态学特征、分子谱、行为和鉴别诊断。
