Solid Form of Systemic EBV+ T-Cell Lymphoproliferative Disease of Childhood
C Barrionuevo, SM Rodriguez-Pinilla, R Pajares, S Montes-Moreno, J Garcia Leon, S Casavilca Zambrano, J Montes Gil, L Sanchez, MA Piris. INEN, Lima, Peru; CNIO, Madrid, Spain
Background: The-systemic-EBV-positive-T-cell-lymphoproliferative-disease-of-childhood is a-poorly -recognized-life-threatening-illness-of-children/young-adults characterized by a-clonal-proliferation-of-EBV-infected-T-cells with-an-activated-cytotoxic-phenotype. We report 6 pediatric-patients from Peru with tumoral-involvement-of-peripheral-lymph-nodes-by-EBV+-peripheral-T-cell-Lymphoma(EBV-PTCL) that could be considered a solid-form-of-this-disease.
Design: 6 pediatric-EBV-PTCLs were analyzed for a-large-panel-of-antibodies and clonal-TCR-rearrangements. Combinations of in-situ-hybridization(EBER)/immunohistochemical-staining were done when required. Morphological,clinical,and follow-up data were reviewed.
Results: All were men developing the disease at the median age of 9-years(range,5/12). Most patients debuted with fever,lost of weight,hepatosplenomegaly,peripheral lymphadenopaty and high-LDH-levels. Lymph nodes were partially/totally replaced by a diffuse-pleomorphic-proliferation-of-neoplastic-cells with a variable range of atypia except for one case that resembles a lymphocyte-rich-classical-Hodgkin-lymphoma. EBER-positive-cells were immunoreactive for cytotoxic-markers(6/6),CD3(6/6),CD43(5/6),CD2(6/6),CD7(3/6),CD5(1/6),TCR-BF1(1/6),CD56(2/6),CD25(3/6),CD30(5/6), BCL2(2/5),CD57(1/6),CD15(1/6), high-Ki67 (5/6). No immunoreactivity for ALK,TDT,CD4,CD8,CD10,Foxp3,CD20,PAX5, BCL6 or CXCL13 was seen. LMP-1-EBV and p53 were positive in 3/6 cases, respectively. One-negative-TCR-BF1-case displayed a TCR-delta-clonal-expansion. All patients died rapidly,within a period of 1/19-months. 4/5-patients received systemic-chemotherapy with partial-response in three. Final-death was attributed to hepatic-failure,respiratory-distress or/and sepsis. Data regarding previous EBV-infection was not available.
Conclusions: Pediatric-EBV-PTCL is an aggressive form of EBV-related-lymphoproliferative disease-of-childhood, presenting in endemic areas, that can be considered a solid-form-of-systemic-EBV-positive-T-cell-lymphoproliferative-disease-of-childhood, with cases exhibiting a T-cell-g/d-immunophenotype. They have a dismal prognosis and poor-response to standard-chemotherapeutic-regimens.
Monday, March 22, 2010 1:00 PM
Poster Session II # 138, Monday Afternoon