Hydroa Vacciniforme (HV) Like T-Cell Lymphoma in Mexican Children: A Lymphoproliferative Disease of T- or NK- Cells
F Nagl, P Grube, D Carrasco, M Saez-de-Ocariz, C Duran-McKinster, C Lome-Maldonado, I Bonzheim, F Fend, L Quintanilla-Martinez. University of Tuebingen, Tuebingen, Germany; Instituto Nacional de Pediatria, Mexico City, Mexico; Instituto Nacional de la Nutricion, Mexico City, Mexico
Background: HV like T-cell lymphoma was included in the 2008 WHO classification as a subgroup of EBV-positive T-cell lymphoproliferative disorders of childhood. It affects mainly children and adolescents from Asia and Latin America. The neoplastic cells are mostly cytotoxic CD8+T-cells and rarely NK-cells. Patients present clinically with a papulo-vesicular rash in sun-exposed areas, followed by ulceration and vacciniform scars. The relation between HV like-T-cell lymphoma and hypersensitivity to mosquito bites, both EBV+ disorders, is unclear. The aim of this study was to analyze phenotypically and molecularly 6 cases clinically diagnosed as HV.
Design: Material and Methods: Paraffin sections were stained with antibodies against CD 3, CD 4, CD 8, CD 20, CD 30, CD56 and TIA-1. EBV-encoded small nuclear RNA (EBER) was analyzed by in-situ-hybridisation. TCRγ gene rearrangement was analyzed by PCR.
Results: Six Mexican cases, 5 males and 1 female were included. The mean age of the patients was 8 years (range 2 - 13 years). All presented with HV-like skin lesions, one had hypersensitivity to insect bites. In two cases the neoplastic cells revealed a NK–cell phenotype (CD 56+, TIA-1+ and CD 30+), whereas four cases featured a cytotoxic T-cell phenotype (CD 3+, CD 8+ and CD4-). All cases were EBER+. The three CD8+ cases analyzed showed monoclonal TCRγ gene rearrangement. The two cases with NK-cell phenotype were polyclonal. One of the patients with NK-cell phenotype progressed to systemic NK/T cell lymphoma and died of disease 4 years after initial diagnosis. One case was lost to follow-up, and 4 cases are alive with wax and wane disease, after a mean follow-up of 7 years (range 2 - 11 years). All cases were treated with Thalidomide and 3 cases with Chemotherapy.
Conclusions: In this study, 1) we demonstrated that cases clinically presenting as HV may show a CD8+ or a NK-cell phenotype. 2) Cases with a CD8+ phenotype show monoclonal TCRγ gene rearrangement from the beginning. 3) The two cases with a NK phenotype showed strong expression of CD30+. 4) Rarely these cases progress to a systemic disease. 5) There is a strong male predominance.
Monday, March 22, 2010 1:00 PM
Poster Session II # 139, Monday Afternoon