Pathology Unit, Department of Mental and Physical Health and Preventive Medicine, University of Campania "Luigi Vanvitelli", Naples, Italy.
Dermatology Unit, Department of Mental and Physical Health and Preventive Medicine, University of Campania "Luigi Vanvitelli", Naples, Italy.
J Cutan Pathol. 2021 Apr;48(4):535-540. doi: 10.1111/cup.13795. Epub 2020 Sep 9.
Primary cutaneous B-cell lymphomas are a heterogeneous group of lymphoid neoplasms primarily occurring in the skin. Although most cases are represented by primary cutaneous follicle center cell lymphoma, primary cutaneous marginal zone lymphoma and leg-type diffuse large B-cell lymphoma, other diffuse large B-cell lymphomas and B-cell lymphoblastic lymphoma may rarely present primarily in the skin. In this setting, the presence of histopathologic and immunohistochemical features of cellular immaturity is exceedingly rare and may represent a diagnostic challenge. We present the first case of a primary cutaneous diffuse large B-cell lymphoma characterized by diminished expression of CD45, expression of TdT and rearrangement of MYC gene. The differential diagnosis mainly included B-cell lymphoblastic lymphoma, and required the genetic analysis of heavy chain (IGH) gene rearrangements.
原发性皮肤 B 细胞淋巴瘤是一组异质性的淋巴肿瘤，主要发生在皮肤。虽然大多数病例表现为原发性皮肤滤泡中心细胞淋巴瘤、原发性皮肤边缘区淋巴瘤和腿型弥漫性大 B 细胞淋巴瘤，但其他弥漫性大 B 细胞淋巴瘤和 B 细胞淋巴母细胞淋巴瘤也可能很少见地首先发生在皮肤。在这种情况下，出现细胞不成熟的组织病理学和免疫组织化学特征极为罕见，可能构成诊断挑战。我们报告首例原发性皮肤弥漫性大 B 细胞淋巴瘤病例，其特征为 CD45 表达减少、TdT 表达和 MYC 基因重排。主要鉴别诊断包括 B 细胞淋巴母细胞淋巴瘤，需要对重链（IGH）基因重排进行遗传分析。
