Characteristics of Cutaneous Marginal Zone Lymphomas With Marked Plasmacytic Differentiation and a T Cell-Rich Background

• Published in: American journal of clinical pathology 2010, Vol.133 (1), p.59-69
• Main Authors: TURBINER GEYER, Julia, FERRY, Judith A, LONGTINE, Janina A, FLOTTE, Thomas J, HARRIS, Nancy L, ZUKERBERG, Lawrence R
• Format: Article
• Language: English
• Subjects: Adult; Aged; Aged, 80 and over; Biological and medical sciences; Cell Transformation, Neoplastic; DNA, Neoplasm - analysis; Female; Gene Rearrangement, B-Lymphocyte, Heavy Chain - genetics; Hematologic and hematopoietic diseases; Humans; In Situ Hybridization; Investigative techniques, diagnostic techniques (general aspects); Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymph Nodes - pathology; Lymphoma, B-Cell, Marginal Zone - genetics; Lymphoma, B-Cell, Marginal Zone - immunology; Lymphoma, B-Cell, Marginal Zone - pathology; Male; Medical sciences; Middle Aged; Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques; Plasma Cells - immunology; Plasma Cells - pathology; Skin Neoplasms - genetics; Skin Neoplasms - immunology; Skin Neoplasms - pathology; T-Lymphocytes - immunology; T-Lymphocytes - pathology; Young Adult
• ISSN: 0002-9173; 1943-7722
• 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.