Current Management of Juvenile Myelomonocytic Leukemia and the Impact of RAS Mutations

@article{Yoshida2012CurrentMO,
  title={Current Management of Juvenile Myelomonocytic Leukemia and the Impact of RAS Mutations},
  author={Nao Yoshida and Sayoko Doisaki and Seiji Kojima},
  journal={Pediatric Drugs},
  year={2012},
  volume={14},
  pages={157-163}
}
Juvenile myelomonocytic leukemia (JMML) is a rare clonal myelodysplastic/myeloproliferative disorder that affects young children. It is characterized by hypersensitivity of JMML cells to granulocyte-macrophage colony-stimulating factor (GM-CSF) in vitro. The pathogenesis of JMML seems to arise from constitutional activation of the GM-CSF/RAS (a GTPase) signaling pathway, a result of mutations in RAS, NF1, PTPN11, and CBL that interfere with downstream components of the pathway. Most patients… Expand
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