title: A 2-year-old with atypical CML with a t(5;12)(q33;p13) treated successfully with imatinib mesylate.
abstract: Atypical chronic myelogenous leukemia (aCML) is a myelodysplastic/myeloproliferative disorder that usually occurs in older adults. Here we report a pediatric patient with aCML and a t(5;12)(q33;p13) with a corresponding fusion gene ETV6-PDGFRB. Because the PDGFRB tyrosine kinase is one of the known targets of tyrosine kinase inhibitors, this patient achieved cytogenetic and molecular remission with treatment with imatinib mesylate (formerly STI571; now Gleevec in the United States and Glivec in Europe). This case illustrates one of many myelodysplastic/myeloproliferative disorders that can be treated with this particular tyrosine kinase inhibitor.
authors.affiliation: Department of Pediatric Hematology/Oncology, Golisano Children's Hospital at Strong, University of Rochester Medial Center, Box 777, 601 Elmwood Avenue, Rochester, NY 14642, USA.
authors.forename: Brenda
authors.initials: B
authors.lastname: Wittman
authors.forename: John
authors.initials: J
authors.lastname: Horan
authors.forename: Joanna
authors.initials: J
authors.lastname: Baxter
authors.forename: John
authors.initials: J
authors.lastname: Goldberg
authors.forename: Raymond
authors.initials: R
authors.lastname: Felgar
authors.forename: Erin
authors.initials: E
authors.lastname: Baylor
authors.forename: Bean
authors.initials: B
authors.lastname: Cromwell
authors.forename: Nicholas
authors.initials: N
authors.lastname: Cross
authors.forename: John M
authors.initials: JM
authors.lastname: Bennett
mesh_terms: D001549:Benzamides; D002675:Child, Preschool; D002881:Chromosomes, Human, Pair 12; D002895:Chromosomes, Human, Pair 5; D005260:Female; D006801:Humans; D000068877:Imatinib Mesylate; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000188:drug therapy* / Q000235:genetics* / Q000473:pathology; D015514:Oncogene Proteins, Fusion / Q000037:antagonists & inhibitors; D010879:Piperazines / Q000627:therapeutic use*; D011743:Pyrimidines / Q000627:therapeutic use*; D012074:Remission Induction; D014178:Translocation, Genetic; D020797:Receptor, Platelet-Derived Growth Factor beta; D000097764:ETS Translocation Variant 6 Protein
chemical_list: D001549:Benzamides; D000068877:Imatinib Mesylate; D015514:Oncogene Proteins, Fusion; D010879:Piperazines; D011743:Pyrimidines; C464679:ETV6-PDGFRB fusion protein, human; D020797:Receptor, Platelet-Derived Growth Factor beta; D000097764:ETS Translocation Variant 6 Protein