title: A unique presentation of an Epstein-Barr virus-associated natural killer/T-cell lymphoproliferative disorder in a white male adolescent.
abstract: BACKGROUND
  Extranasal natural killer (NK)/T-cell lymphoma and aggressive NK-cell leukemia are strongly associated with Epstein-Barr virus (EBV) and most often occur in middle-aged individuals. Overlap between these 2 diagnoses is rare. In addition, pathologic findings for these 2 diagnoses are typically notable for necrosis, apoptosis, angioinvasion, and angiodestruction.
  
  
  METHODS
  We describe a 15-year-old male adolescent who had painful subcutaneous nodules and plaques over his anterior thighs, shins, and lower abdomen while receiving anti-tumor necrosis factor therapy with infliximab. He also was noted to have pulmonary nodules, liver nodules, hepatosplenomegaly, thrombocytopenia, and transaminitis. A skin biopsy revealed atypical small to intermediate-sized EBV-positive lymphoid cells of NK-cell origin infiltrating the subcutaneous adipose tissue, mimicking subcutaneous T-cell lymphoma. Similar atypical EBV-positive lymphocytes were noted in the bone marrow, liver, stomach, and colon. This patient had a rapidly fatal disease course.
  
  
  CONCLUSIONS
  We report a unique clinical and histological presentation most consistent with an extranasal NK/T-cell lymphoma and aggressive NK-cell leukemia overlap, although our case may represent a disease entity completely new to the literature. In addition, we report the first case to our knowledge of an EBV-positive NK/T-cell lymphoma developing in the setting of tumor necrosis factor inhibitor therapy.
authors.affiliation: Department of Dermatology, University of Utah, Salt Lake City, UT 84132, USA. rika.summers@hsc.utah.edu
authors.forename: Erika
authors.initials: E
authors.lastname: Summers
authors.forename: George
authors.initials: G
authors.lastname: Samadashwily
authors.forename: Scott R
authors.initials: SR
authors.lastname: Florell
mesh_terms: D000293:Adolescent; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D003937:Diagnosis, Differential; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D017809:Fatal Outcome; D004854:Herpesvirus 4, Human; D006801:Humans; D000069285:Infliximab; D007945:Leukemia, Lymphoid / Q000473:pathology; D016399:Lymphoma, T-Cell / Q000473:pathology / Q000821:virology*; D008232:Lymphoproliferative Disorders / Q000473:pathology / Q000821:virology*; D008297:Male; D055611:Natural Killer T-Cells / Q000473:pathology / Q000821:virology*; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors / Q000627:therapeutic use
chemical_list: D000893:Anti-Inflammatory Agents; D000911:Antibodies, Monoclonal; D014409:Tumor Necrosis Factor-alpha; D000069285:Infliximab