Clinicopathological analysis of systemic EB virus-positive T-cell lymphoproliferative disease in childhood

Abstract

Objective To investigate the clinicopathological and immunophenotypic features of systemic EB virus (EBV)-positive T-cell lymphoproliferative disease of childhood (CSEBV+T-LPD). Methods The clinicopathological features of 11 patients with CSEBV+ T-LPD were reviewed by using immunohistochemistry, fluorescence in situ hybridization, gene rearrangement method. The follow-up data were collected. Results Of the 11 patients, 5 cases were male and 6 cases were female. The median age was 13 years old (3-19 years old) and the disease course was 6 months (3-25 months). 2 male and 2 female patients were diagnosed with lymphoma. The median age was 15 years old, and the median disease course was 4.5 months. The major symptoms included continued fever (10/11), lymphadenectasis (10/11), splenomegaly (7/11), hepatomegaly (4/11). Histopathology: 4 cases of lymphoma showed lymphocyte flaky or diffuse hyperplasia, and cell volume was too large, isolated hyperplasia cells; 7 cases of benign lesions were mixed cells, and proliferation of lymphocytes were scattered with moderate big or much bigger volume.Immunophenotyping: all biopsies exhibited prominent T cell proliferation, as defined by immunohistochemical staining of CD3e. 2 cases of CD8 single positive in 4 lymphoma patients, 1 case of CD8 single positive in 7 cases of benign lesions. T-cell antigen loss was detected in all the patients, while only 1 loss case was found in patients with benign lesions. All cases were positive for EBV-EBER. T-cell receptor (TCR) gene detection: 3 lymphoma patients with TCR gene cloning rearrangement, 1 case of benign lesions with cloned rearrangement. Pathology classification: 4 cases of lymphoma were A3, 1 case of benign lesions was A2, 6 cases of benign lesions were A1. Conclusions CSEBV+T-LPD comprises a wide spectrum of diseases. The diagnosis should combine with clinical features, pathological morphology, immune phenotype, EBV-EBER in situ hybridization and TCR gene detection. Furthermore, elderly patients, with single hyperplasia of cells, diffuse or flake, CD8 single positive, T cell antigen loss especially CD5, high expression of EBV-EBER, TCR clonal rearrangement, A3 pathological grading, may play a key role in differentiating benign and malignant lesions. Key words: Epstein-Barr virus; T-cell lymphoproliferative disorders; Lymphoma, T-cell; In situ hybridization; T-cell receptor gene rearrangements