title: Lethal Epstein-Barr virus associated NK/T-cell lymphoma with primary manifestation in the conjunctiva.
abstract: BACKGROUND
  Lethal midline granulomas (LMG) are very rare angiocentric NK/T-cell lymphomas in association with Epstein-Barr virus. LMG are reported mainly in East Asia occurring in immune compromised patients.
  
  
  METHODS
  A 41-year old male patient presented with a conjunctival swelling of his upper left eyelid. The lesion had increased over a period of 2 months despite topical corticosteroid treatment. Conjunctival biopsy revealed a highly malignant, CD3 + and BCL2 + extranodal T-cell lymphoma with features of an NK/T-cell origin (CD56 +, TIA + TCR-rearrangement: germline). All lymphoma cells were positive for Epstein-Barr virus RNA. The proliferation rate was highly elevated at 100 %.
  
  
  RESULTS
  Systemic 1 (st) cycle chemotherapy with cyclophosphamide, doxorubicin, vincristin and prednisone resulted in a complete remission of the swelling within 4 days. However, one week later a massive conjunctival tumour reappeared with only partial regression after combined chemo- and radiotherapy. The patient died within a month because of untreatable pancytopenia due to malignant bone marrow infiltration.
  
  
  CONCLUSIONS
  LMG is a rare but highly malignant Epstein-Barr virus associated NK/T-cell lymphoma that can occur in healthy, immune competent Caucasians. This is the first reported case of an LMG in an immune-competent Caucasian patient with primary ocular manifestation. The LMG has a high mortality rate despite systemic treatment and can be lethal within a few months or even weeks.
authors.affiliation: Department of Ophthalmology, University Eye Hospital Zurich, Zurich, Switzerland. stefanwidmer@yahoo.com
authors.forename: S
authors.initials: S
authors.lastname: Widmer
authors.forename: M
authors.initials: M
authors.lastname: Tinguely
authors.forename: F
authors.initials: F
authors.lastname: Egli
authors.forename: M A
authors.initials: MA
authors.lastname: Thiel
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001706:Biopsy; D017252:CD3 Complex / Q000032:analysis; D003228:Conjunctiva / Q000473:pathology; D003230:Conjunctival Neoplasms / Q000175:diagnosis* / Q000473:pathology; D020031:Epstein-Barr Virus Infections / Q000175:diagnosis* / Q000473:pathology; D005142:Eyelid Neoplasms / Q000175:diagnosis* / Q000473:pathology; D005143:Eyelids / Q000473:pathology; D017809:Fatal Outcome; D006103:Granuloma, Lethal Midline / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy; D006801:Humans; D007694:Killer Cells, Natural / Q000473:pathology*; D016411:Lymphoma, T-Cell, Peripheral / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy; D009364:Neoplasm Recurrence, Local / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000032:analysis; D018714:Radiotherapy, Adjuvant; D013601:T-Lymphocytes / Q000473:pathology*
chemical_list: D017252:CD3 Complex; D019253:Proto-Oncogene Proteins c-bcl-2