EBV-positive Mucocutaneous Ulcer: A Case Report and Review of the First 100 Published Cases

Publication date: Available online 13 October 2018Source: Clinical Lymphoma Myeloma and LeukemiaAuthor(s): Ryan B. Sinit, Kathleen L. Horan, Russell K. Dorer, David M. AboulafiaAbstractIn 2016, the World Health Organization provisionally classified Epstein-Barr virus (EBV)-positive mucocutaneous ulcer (EBVMCU) as a lymphoid neoplasm under the subcategory of mature B-cell neoplasms. EBVMCU is manifested as a well-circumscribed ulcer, typically unifocal, and of the oropharynx, gastrointestinal tract, or skin. Immunohistochemical studies of these ulcers reveal monoclonal B-immunoblasts staining positively for CD20, CD30, EBER, MUM-1, OCT-2, and PAX-5 and staining variably for BCL-6, CD15, CD45, and CD79a. As EBV-associated lymphoproliferative disorders occur when EBV-infected cells expand while escaping immunosurveillance, EBVMCU is most commonly associated with medication-induced immunosuppression, advanced age-associated immunosenescence, and primary or acquired immunodeficiencies. Herein, we describe an unusual case of EBVMCU in a woman with an antecedent history of asymptomatic EBV infection and a lifelong history of sinopulmonary infections. She received radiation therapy and several years later was diagnosed with diffuse large B-cell lymphoma (DLBCL). The association of chronic antigenic stimulation and underlying subtle immunodeficiency are etiologic links between EBVMCU and DLBCL. We also review the literature of the first 100 reported cases of EBVMCU, inclusive of our o...
Source: Clinical Lymphoma Myeloma and Leukemia - Category: Cancer & Oncology Source Type: research