Abstract
Background: Epstein Barr Virus (EBV) is associated with different kinds of tumors. In the present study we tried to understand its role in pediatric MDS of a 4-year-old girl with EBV infection and MDS with refractory anemia and monosomy 7. Procedures: The work-up included: hematological tests; serology for IgM, IgG antibodies to EBV; PCR for EBV; cytogenetics of bone marrow (BM), and FISH analysis of BM and blood; immunohistochemistry-LMP1 expression on BM smears. Results: Hematological follow up showed constant mild dysplastic changes mostly in the erythroid lineage. PCR for EBV showed positive results in the nasopharygeal smears as well as in blood 15 weeks after disease onset. Cytogenetic analysis showed monosomy7 inall the mitoses of BM sample. Fluorescence in situ hybridization (FISH) showed monosomy 7% in 57% of the cells, followed by a decreased tendency in the percentage of monosomy 7 cells in both BM and blood. Immunohistochemistry for EBV-latent membrane protein 1 (LMP-1) on the patient’s BM smears, 21 weeks post disease onset, showed 9% positive cells, 80% of them carried monosomy 7. Conclusion: The parallel occurrence of the EBV infection and MDS, as well as the continuous EBV PCR positive and monosomy 7, support the possibility that they are related.
Highlights
Myelodysplastic syndrome (MDS) represents a heterogeneous clonal hematopoetic stem cell disorder characterized by ineffective hematopoiesis and increased risk of transformation to MDS-related leukemia
Cytogenetic analysis of the first bone marrow sample revealed monosomy 7 as a sole aberration in all the analyzed dividing cells; Fluorescence in situ hybridization (FISH) analysis revealed that only 57% of the cells carry monosomy 7
We described here a 4-year-old girl suffering from simultaneous acute Epstein Barr Virus (EBV) infection and refractory anemia MDS with chromosome 7 monosomy
Summary
Myelodysplastic syndrome (MDS) represents a heterogeneous clonal hematopoetic stem cell disorder characterized by ineffective hematopoiesis and increased risk of transformation to MDS-related leukemia. In the present study we tried to understand its role in pediatric MDS of a 4-year-old girl with EBV infection and MDS with refractory anemia and monosomy 7. Procedures: The work-up included: hematological tests; serology for IgM, IgG antibodies to EBV; PCR for EBV; cytogenetics of bone marrow (BM), and FISH analysis of BM and blood; immunohistochemistry-LMP1 expression on BM smears. PCR for EBV showed positive results in the nasopharygeal smears as well as in blood 15 weeks after disease onset. Immunohistochemistry for EBV-latent membrane protein 1 (LMP-1) on the patient’s BM smears, 21 weeks post disease onset, showed 9% positive cells, 80% of them carried monosomy 7. Conclusion: The parallel occurrence of the EBV infection and MDS, as well as the continuous EBV PCR positive and monosomy 7, support the possibility that they are related
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