Hydroa Vacciniforme-Like Cutaneous T-Cell Lymphoma. Report of a Series of 17 Cases
SM Rodriguez-Pinilla, C Barrionuevo, R Pajares, S Montes-Moreno, J Garcia Leon, S Casavilca Zambrano, J Montes Gil, MA Piris. CNIO, Madrid, Spain; INEN, Lima, Peru
Background: Hydroa-vacciniforme-like-lymphoma is an Epstein-Barr-virus(EBV)-positive-T-cell lymphoproliferative-disease-of-childhood recognized by the WHO that remains poorly characterized. We report 17-peruanian cases in an attempt to better characterize/understand this entity.
Design: 17-cases-of-hydroa-like-T-cell-lymphomas were analyzed for a-large-panel-of-antibodies. Combinations of in-situ-hybridization(EBER) and immunohistochemical-staining were done in all cases. Morphological, clinical, and follow-up data were reviewed.
Results: The disease started at the median age of 8.7 years(range, 1-17) in 9 boys and 5 females. We did not have data regarding the previous EBV-status of the patients. EBER-positive-cell with slight/moderate-atypia were seen in the dermis surrounding superficial vessels and adnexa. Intensity of the infiltrate varied from case to case . Epidermotropism, involvement of the hypodermis or skeletal muscle could be seen. Hydroa-changes were frequently observed. EBER-positive-cells were positive for T-cell-cytotoxic-markers in all cases as well as for cytoplasmic CD3ε(17/17), CD2(13/17), CD5(7/17), CD7(6/16), TCR-βFI(3/16), CD8(9/17), CD4(0/17), CD56(5/16), CD57(1/16), PD1(0/15), FOXP3(0/15), CD30(4/17), CD25(1/16), CD43(11/16). Eight cases had a high proliferation-index(ki67) while EBV-LMP1-was-positive in only three . All were sun-exposed-skin-lesions that suffered an intermittent course changing from edema, to blistering, ulceration and scarring. Five cases suffered progression with peripheral lymph node involvement while the rest remained as a recurrent cutaneous disorder. No correlation was found between the ki67-expression and lymph node involvement, although 3/5 cases showed CD30-positivity. Only 4 patients remain alive after a median follow-up of 73 months(range 380-6). 10 patients died in an average period of 7.8 months after starting treatment, 6 of concurrent infections and 7 due to progression.
Conclusions: Hydroa-like-T-cell lymphoma is a distinctive lymphoproliferative-disorder, with specific morphological and immunophenotypic features. In spite of the tendency to remain in the skin, most of the cases show a dismal prognosis, with poor response to standard chemotherapeutic regimens
Monday, March 22, 2010 1:30 PM
Platform Session: Section B, Monday Afternoon