Journal
LEUKEMIA
Volume 26, Issue 11, Pages 2384-2389Publisher
NATURE PUBLISHING GROUP
DOI: 10.1038/leu.2012.109
Keywords
tyrosine kinase; sorafenib; CMML
Categories
Funding
- Institut National du Cancer (INCa)
- Association pour la Recherche contre le Cancer (ARC)
- CITTIL (Cooperacion de investigacion transpirenaica en la terapia innovadora de la leucemia)
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Myeloproliferative neoplasms are frequently associated with aberrant constitutive tyrosine kinase (TK) activity resulting from chimaeric fusion genes or point mutations such as BCR-ABL1 or JAK2 V617F. We report here the cloning and functional characterization of two novel fusion genes BCR-RET and FGFR1OP-RET in chronic myelomonocytic leukemia (CMML) cases generated by two balanced translocations t(10; 22)(q11;q11) and t(6;10)(q27;q11), respectively. The two RET fusion genes leading to the aberrant activation of RET, are able to transform hematopoietic cells and skew the hematopoietic differentiation program towards the monocytic/macrophage lineage. The RET fusion genes seem to constitutively mimic the same signaling pathway as RAS mutations frequently involved in CMML. One patient was treated with Sorafenib, a specific inhibitor of the RET TK function, and demonstrated cytological and clinical remissions.
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