Cutaneous Presentation of Mantle Cell Lymphoma

Abstract

© 2010 The Authors. doi: 10.2340/00015555-0886 Journal Compilation © 2010 Acta Dermato-Venereologica. ISSN 0001-5555 Mantle cell lymphoma (MCL) is a lymphoid malignancy of B-cells of the mantle zone or primary lymphoid follicle of lymph nodes. It is a moderately aggressive B-cell non-Hodgkin’s lymphoma (NHL). MCL represents only 6% of all NHL (1). MCL is listed in the new World Health Organization (WHO) European Organization for Research and Treatment for Cancer (EORTC) classification of cutaneous lymphomas that can secondarily involve the skin (2). According to this classification, “primary cutaneous lymphoma” refers to cutaneous T-cell lymphomas (CTCLs) and cutaneous B-cell lymphomas (CBCLs) that present in the skin with no evidence of extracutaneous disease at the time of diagnosis (2). The current WHO guidelines for the diagnosis of MCL rely on morphological examination and immunophenotyping with demonstration of cyclin D1 protein overexpression and/or the t(11;14)(q13;q32) translocation for confirmation (3). There are two primary B-cell lymphomas that are more common than MCL; cutaneous marginal zone Bcell lymphoma, and primary cutaneous follicle centre lymphoma. The former is an indolent cutaneous B-cell lymphoma derived from post-germinal centre cells and characterized by a proliferation of small lymphocytes, lymphoplasmo cytoid cells and plasma cells with monotypic cytoplasmic immunoglobulin. Follicle centre cell lymphoma is derived from follicle centre cells, consisting of a mixture of centrocytes and centroblasts (2, 4). Immunophenotyping assists in the differential diagnosis of these cutaneous lymphomas. Skin involvement in MCL is very rare (1). We describe here a case of MCL involving the skin as the first manifestation of the disease, and we review the clinical features of reported cases of MCL with skin lesions. CASE REpORT