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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 wereor= 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.

This study was aimed to explore the effect of ex vivo chemical modification of graft cells with methoxy polyethylene glycol (mPEG) on graft versus host disease (GVHD) after haploidentical stem cell transplantation in neonatal mice and its influence on activity of the stem cells. The modified and non-modified spleen cells of adult CB6F1 mice were injected into the abdominal cavity of neonatal BALB/c mice with 5x10(6) spleen cells per mouse, and GVHD were measured by spleen index (SI). Furthermore, the modified and non-modified mixture of bone marrow and spleen cells (BMS) were transplanted to haploidentical lethally irradiated adult BALB/c mice via tail vein with 2x10(5) BMS per mouse, and the colony forming units of spleens (CFU-S) were counted on the eighth day after irradiation. The results indicated that SI1 in modification group were lower than that in non-modification group, and SI2 in modification group was1.3, showing that GVHD in modification group were less severe. The numbers of CFU-S formed in both modification group and non-modification group were not significantly different (p0.05), indicating that the activity of the stem cells were not affected by mPEG modification. In conclusion, the modification of graft cells with mPEG alleviates GVHD after haploidentical stem cell transplantation in neonatal mice, and do not influence the activity of the stem cells.

The aim of this study was to investigate the clinical, pathological and biological features of biphenotypic acute leukemia. The morphology of tumor cells was observed by bone marrow examination; the immunophenotype was assayed by flow cytometry and immunohistochemistry; the chromosomal aberrations were detected by conventional chromosomal analysis and RT-multiplex nested PCR. The results showed that extramedullary skin lesions and myelodysplasia occurred before the onset of overt disease. At the time of diagnosis, this case had more than 30% blasts in bone marrow with meningeal involvement. Large-sized tumor cells predominated morphologically over other cells. Flow cytometry revealed the co-expression of myeloid antigens (cMPO, CD33 and CD117) and T-lymphoid antigens (cCD3, CD5, CD7, dual expression of CD4 and CD8). Immunohistochemical staining showed that CD43 and CD99 were strong positive which define the earliest hematopoietic progenitors. Partial tandem duplication of the MLL gene could be detected with normal cytogenetic method. All above-mentioned results led to the diagnosis of biphenotypic acute leukemia. It is concluded that the biphenotypic acute leukemia is an uncommon type of leukemia which may be preceded by myelodysplastic syndrome and has aggressive clinical and biological behavior. Immunophenotype, cytogenetics and molecular analysis can contribute to early diagnosis of BAL and evaluation of prognosis.