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Magaña, M; Massone, C; Magaña, P; Cerroni, L.
Clinicopathologic Features of Hydroa Vacciniforme-Like Lymphoma: A Series of 9 Patients.
Am J Dermatopathol. 2016; 38(1):20-25 Doi: 10.1097/DAD.0000000000000385
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Leading authors Med Uni Graz
Cerroni Lorenzo
Co-authors Med Uni Graz
Massone Cesare
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Abstract:
Hydroa vacciniforme-like lymphoma is a recently recognized cutaneous T-cell lymphoma associated with Epstein-Barr virus. The disease is observed in children of Latin American or Asian ethnicity. The authors report the clinical, histopathological, and immunophenotypical features of 9 new Mexican patients (M:F = 2:1; mean age, 14.5 years; median age, 13.3 years; age range, 4-27 years), expanding on previous observations of this elusive disease. The most common clinical aspects were persistent facial edema with necroses and pitted scars. Histopathological analyses revealed variably dense lymphoid infiltrates with common angiodestructive features. Neoplastic cells expressed CD3 and cytotoxic markers in all cases and were constantly positive for Epstein-Barr virus (EBER-1). Expression of other markers was variable. Follow-up data revealed that all patients died within 6 months or less, thus showing a very aggressive course with poor prognosis.
Find related publications in this database (using NLM MeSH Indexing)
Adolescent -
Adult -
CD3 Complex - analysis
Child -
Child, Preschool -
Cicatrix - pathology
Cicatrix - virology
Edema - pathology
Edema - virology
Epstein-Barr Virus Infections - complications
Extremities - pathology
Face - pathology
Facial Neoplasms - chemistry
Facial Neoplasms - pathology
Facial Neoplasms - virology
Female -
Humans -
Hydroa Vacciniforme - pathology
Hydroa Vacciniforme - virology
Immunohistochemistry -
Lymphoma, T-Cell, Cutaneous - chemistry
Lymphoma, T-Cell, Cutaneous - pathology
Lymphoma, T-Cell, Cutaneous - virology
Male -
Mexico -
Necrosis - pathology
Necrosis - virology
Prognosis -
Torso - pathology
Young Adult -

Find related publications in this database (Keywords)
hydroa-like lymphoma
hydroa vacciniforme-like cutaneous T-cell lymphoma
angiocentric cutaneous T-cell lymphoma of childhood
Epstein-Barr virus
hydroa vacciniformis
cutaneous T-cell lymphoma
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