title: Hydroa vacciniforme-like lymphoma: a chronic EBV+ lymphoproliferative disorder with risk to develop a systemic lymphoma.
abstract: Hydroa vacciniforme-like lymphoma (HVLL) is an Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disorder of childhood that occurs mainly in Central and South America and Asia. We present the clinicopathological features of 20 Mexican children with HVLL with a median age of 8 years at diagnosis (range, 1-15). All patients presented with skin lesions involving sun-exposed areas, but not exclusively. Fever, lymphadenopathy, and hepatosplenomegaly were often observed. Most patients were treated with immunomodulators and/or immunosuppressive agents, resulting in temporary remission. For 13 patients follow-up was available for a median of 3 years (range, 1 month-13 years). Three patients with long follow-up (9-13 years) are alive with disease. Four patients died, 2 after developing systemic lymphoma. Histologically, the skin showed a predominantly angiocentric and periadnexal Epstein-Barr early RNA+ lymphoid infiltrate with variable atypia and subcutaneous involvement. Fifteen patients showed a T-cell phenotype (12, αβ; 2, γδ; 1, silent phenotype) and monoclonal T-cell receptor-γ rearrangements, whereas 6 exhibited a natural killer (NK)-cell phenotype. Four patients had hypersensitivity to mosquito bites. One patient showed both phenotypes. HVLL is an EBV-associated lymphoproliferative disorder of αβ-, γδ-, or NK-cell phenotype with a broad clinical spectrum, usually prolonged clinical course, and risk for progression to systemic disease.
authors: Institute of Pathology and Comprehensive Cancer Center, Eberhard-Karls-University of Tübingen, Tübingen, Germany;
authors: Leticia
authors: L
authors: Quintanilla-Martinez
authors: Cecilia
authors: C
authors: Ridaura
authors: Florian
authors: F
authors: Nagl
authors: Marimar
authors: M
authors: Sáez-de-Ocariz
authors: Carola
authors: C
authors: Durán-McKinster
authors: Ramon
authors: R
authors: Ruiz-Maldonado
authors: Georgia
authors: G
authors: Alderete
authors: Peter
authors: P
authors: Grube
authors: Carmen
authors: C
authors: Lome-Maldonado
authors: Irina
authors: I
authors: Bonzheim
authors: Falko
authors: F
authors: Fend
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D005260:Female; D005500:Follow-Up Studies; D004854:Herpesvirus 4, Human / Q000187:drug effects / Q000235:genetics / Q000378:metabolism; D006801:Humans; D006837:Hydroa Vacciniforme / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D007150:Immunohistochemistry; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D017403:In Situ Hybridization; D007223:Infant; D016410:Lymphoma, T-Cell, Cutaneous / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D008232:Lymphoproliferative Disorders / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D008297:Male; D008800:Mexico; D012367:RNA, Viral / Q000235:genetics; D011948:Receptors, Antigen, T-Cell / Q000235:genetics; D013256:Steroids / Q000627:therapeutic use; D013601:T-Lymphocytes / Q000187:drug effects / Q000378:metabolism / Q000821:virology; D013792:Thalidomide / Q000627:therapeutic use; D014764:Viral Proteins / Q000378:metabolism
chemical_list: D007166:Immunosuppressive Agents; D012367:RNA, Viral; D011948:Receptors, Antigen, T-Cell; D013256:Steroids; D014764:Viral Proteins; D013792:Thalidomide