[Characteristics of chronic active Epstein-Barr virus infection-associated hematological disorders in children]

The aim of this study was to analyze characteristics of chronic active Epstein-Barr virus (CAEBV) infection associated hematological disorders in children. Clinical characteristics were summarized; the morphology of hematopoietic cells in bone marrow was observed by microscopy; the lymphocyte subpopulations were analyzed by flow cytometry; the immunophenotype of liver biopsies was assayed by immunohistochemistry; EBV-related antibodies were measured by ELISA; serum EBV-DNA loads were detected by real-time quantitative PCR; EBV-encoded small RNA 1-positive cells in peripheral blood mononuclear cells were identified by in situ hybridization. The results indicated that the clinical manifestations in patients included persistent or recurrent fever, hepatosplenomegaly, liver dysfunction, anemia, thrombocytopenia, systemic inflammatory reaction. Bone marrow presented as hypocellularity, dysmaturation, myelodysplasia and hemophagocytosis. CD8(+) cell high counts were demonstrated in all 4 patients, one of them developed into a T cell lymphoma. Serum EBV-DNA load was 3.26 x 10(3) copies/ml in one patient, EBER1(+) cells were detected at a frequency of 1.7% in PBMNCs from another patient; the titers of IgG to EBV-VCA were >or= 1:5120 in the rest 2 patients. All 4 patients described above were diagnosed as CAEBV infection. In conclusion, the immune-related cytopenia, macrophage activation syndrome and lymphoproliferative disorders are characteristics of CAEBV infection associated hematological disorders in these 4 children patients.