Juvenile myelomonocytic leukaemia JML

• Clonal disorder arising in pluripotent stem cell causing selective hypersensitivity to GM-CSF due to dysregulated signal transduction through Ras pathway.

• Affects infants and young children usually <5 years of age.

• Marked hepatosplenomegaly, neutrophilia and monocytosis, anaemia and thrombocytopenia.

• No Philadelphia chromosome or BCR-ABL fusion gene.

• No consistently effective therapy including allogeneic SCT (relapse rates up to 55%). 5 year survival after allograft 25-40%.

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