Abstract
This study was to determine whether GM-CSF induced WT1 gene expression and to establish an association with markers of proliferation CD71+CD34+ using nPCR and flow cytometry respectively, in samples obtained from 5 newly diagnosed JMML patients. Overtime (day 0 to day 14) there was an insignificant difference in WT1 gene expression and CD71+CD34+ in JMML samples when compared to peripheral blood of normal volunteers (n = 3). Our study suggests that there is a correlation between WT1 gene expression and cellular proliferation and that GMCSF in vitro does not create a significant difference in JMML samples.
Highlights
Juvenile myelomonocytic leukemia (JMML) is a rare hematopoietic malignancy of early childhood with features exhibiting of both myeloproliferative neoplasm (MPN) and myelodysplastic syndrome (MDS) [1]
JMML affects very young children with a significant male predominance. This leukemia clinically presents with marked hepatosplenomegaly together with lymphadenopathy, pallor and skin rash [2]. It is a representative of a clonal disease of pluripotent cell origin and in vitro granulocyte-macrophage colony-stimulating factor (GMCSF) hypersensitivity is the hallmark of JMML [3,4,5,6,7]
There was a difference found on day 14 in JMML samples for WT1 transcription in assessment to the initial day
Summary
Juvenile myelomonocytic leukemia (JMML) is a rare hematopoietic malignancy of early childhood with features exhibiting of both myeloproliferative neoplasm (MPN) and myelodysplastic syndrome (MDS) [1]. JMML affects very young children (median age at diagnosis is 2 years) with a significant male predominance (male:female = 2.5). This leukemia clinically presents with marked hepatosplenomegaly together with lympha-. Denopathy, pallor and skin rash [2]. It is a representative of a clonal disease of pluripotent cell origin and in vitro granulocyte-macrophage colony-stimulating factor (GMCSF) hypersensitivity is the hallmark of JMML [3,4,5,6,7]. WT1 gene expression has been utilized as a diagnostic tool in assessing minimal residual disease (MRD) in varieties of leukemias [9,11,12,13,14,15] and disease progression in MDS [1115]
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