EBV-associated cutaneous NK/T-cell lymphoma: review of a series of 14 cases from peru in children and young adults.
Am J Surg Pathol
; 34(12): 1773-82, 2010 Dec.
Article
en En
| MEDLINE
| ID: mdl-21107082
ABSTRACT
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.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Neoplasias Cutáneas
/
Células Asesinas Naturales
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Linfocitos T
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Linfoma Cutáneo de Células T
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Infecciones por Virus de Epstein-Barr
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Hidroa Vacciniforme
Tipo de estudio:
Risk_factors_studies
Límite:
Adolescent
/
Adult
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Child
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Child, preschool
/
Female
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Humans
/
Male
País/Región como asunto:
America do sul
/
Peru
Idioma:
En
Revista:
Am J Surg Pathol
Año:
2010
Tipo del documento:
Article
País de afiliación:
España