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Juvenile Myelomonocytic Leukemia
Pathophysiology
No Philadelphia chromosome or Bcr-Abl fusion gene exists in association with JMML.1 However, evidence of a novel chromosomal rearrangement associated with the gene encoding PDGFRB has been reported.2 Morerio and colleagues have described a gene rearrangement, t(5;17)(q33;p11.2), resulting in a constitutively active HCMOGT-1-PDGFRB fusion protein in patients with JMML.2 Similarities in the genetic breakpoints of this fusion gene with other previously identified PDGFRB fusion genes known to result in constitutive tyrosine kinase activity and clonal disease suggest that a similar pathogenetic mechanism may be involved.
References:
- National Cancer Institute. Myelodysplastic/Myeloproliferative Diseases (PDQ®): Treatment. Health Professional Version. Available at: http://www.cancer.gov/cancertopics/pdq/treatment/mds-mpd/healthprofessional/allpages. Accessed April 24, 2006.
- Morerio C, Acquila M, Rosanda C, et al. HCMOGT-1 is a novel fusion partner to PDGFRB in juvenile myelomonocytic leukemia with t(5;17)(q33;p11.2). Cancer Res. 2004;64:2649-2651.
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