RESUMO
OBJECTIVE@#To explore the clinical and pathologic features as well as prognosis of systemic EBV-positive T-cell lymphoma in children.@*METHODS@#The clinical data including clinical manifestation, pathologic changes and treatment in 16 patients with children's systemic EBV-positive T-cell lymphoma were analyzed retrospectively, and follow-up of patients were carried out.@*RESULTS@#The 16 cases included 12 males and 4 females with median age of 3.3 years old. It was demonstrated that the clinical and pathological features of the children's systemic EBV-positive T-cell lymphoma were as followed fever, hepatosplenomegaly, cytopenia, lymphadenopathy, and hemophagocytosis in bone marrow or organ. Histologically, the structures of lymph node was normal, partially or completely destoryed. The paracortical zone was expanded with prominent infiltration of small to medium-sized atypical lymphocytes. The major immunophenotypic characteristics were as follows: (1) Almost all biopsies exhibited prominent T cell proliferation. (2) CD3 was expressed in 16 patients (100%, 16/16), CD4 in 5 patients (31.3%, 5/16)，CD5 in 13 patients (81.3%, 13/16)，CD7 was expressed in 11 patients (68.8%, 11/16)，CD8 in 15 patients (93.8%, 15/16)，CD4 and CD8 were expressed in 5 patients (31.3%, 5/16)，CD4 and CD8 double-negative in patients (6.3%, 1/16)，16 patients were CD56 negative (100%, 16/16). (3) TCR gene cloning rearrangement in 16 patients (93.8%, 15/16). (4) EBV-EBER was expressed in 16 patients (100%, 16/16). 11 out of 16 cases died, 1 cese failed to be followed up, 1 case relapsed，and 3 cases survived, reseptively. The media survival time was 4 months.@*CONCLUSION@#Systemic EBV-positive T-cell lymphoma predominantly occurred in childhood and early teen-age, and lacks specific clinic features, usually combined with hemophagocytic syndrome. The confirmed diagnosis requires comprehensive analysis of clinical manifestation, pathomorphology, immunohistochemical detection, EBV-EBER insite hybridization, and TCR gene test. The overall prognosis of the disease is poor and the fatality rate is high.