EBV-associated cutaneous NK/T-cell lymphoma: review of a series of 14 cases from peru in children and young adults.
We have reviewed clinically, morphologically, and immunophenotypically a series of 14 Epstein-Bar virus (EBV)+ cutaneous natural killer cell (NK)/T-cell lymphoma from Peru. Most (11 out of 14) of these cases fit well into the category of Hydroa vacciniforme-like lymphoma (HVLL), but 3 have a different clinical presentation, without facial involvement. In all 14 cases, skin lesions present in both the sun-exposed and nonexposed areas exhibited a slowly progressive relapsing course, changing from edema, to blistering, ulceration, and final scarring. The immunophenotype had a cytotoxic T or NK-cell lineage. The mean time of disease before admission to hospital was 69 months (range, 6 mo to 31 y). Only 2 patients had fever, hepatosplenomegaly, systemic lymphadenopathy, and a high lactate dehydrodenage (LDH) level at the time of diagnosis, whereas 10 had facial swelling. After treatment, only 4 patients remain alive, although with persistent disease. Ten patients died after a mean follow-up of 11.6 months after the initial diagnosis (range, 1 to 32 mo), because of concurrent infections (4 cases), disease progression (4 patients) or both (2 patients). Endemic Epstein-Bar virus (EBV)-positive cutaneous NK/T-cell lymphoproliferative disorders in childhood and early adulthood are characterized by a protracted clinical course, eventually leading to an aggressive phase characterized by concurrent infections and disease progression.
Lymphoma Group, Molecular Pathology Programme, Spanish National Cancer Research Centre, CNIO, Madrid, Spain. smrodriguez@cnio.es
Socorro Maria
SM
Rodríguez-Pinilla
Carlos
C
Barrionuevo
Juan
J
Garcia
Marco T
MT
Martínez
Raquel
R
Pajares
Santiago
S
Montes-Moreno
Sandro
S
Casavilca
Jaime
J
Montes
Francisco
F
Bravo
Mayer
M
Zaharia
Eduardo
E
Zevallos-Giampietri
Lydia
L
Sanchez
Miguel A
MA
Piris
D000293:Adolescent; D000328:Adult; D014408:Biomarkers, Tumor / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D002999:Clone Cells; D020031:Epstein-Barr Virus Infections / Q000276:immunology / Q000401:mortality / Q000473:pathology*; D005260:Female; D004854:Herpesvirus 4, Human / Q000235:genetics / Q000302:isolation & purification; D006801:Humans; D006837:Hydroa Vacciniforme / Q000276:immunology / Q000401:mortality / Q000473:pathology*; D017403:In Situ Hybridization; D007694:Killer Cells, Natural / Q000276:immunology / Q000473:pathology*; D016410:Lymphoma, T-Cell, Cutaneous / Q000276:immunology / Q000401:mortality / Q000473:pathology*; D008297:Male; D010568:Peru / Q000453:epidemiology; D012867:Skin / Q000473:pathology / Q000821:virology; D012878:Skin Neoplasms / Q000276:immunology / Q000401:mortality / Q000473:pathology*; D015996:Survival Rate; D013601:T-Lymphocytes / Q000276:immunology / Q000473:pathology*; D055815:Young Adult
D014408:Biomarkers, Tumor