title: Myeloproliferative disorder with eosinophilia and ETV6-ABL gene rearrangement: efficacy of second-generation tyrosine kinase inhibitors.
abstract: ETV6/ABL is a rare gene rearrangement that has rarely been detected in Philadelphia-negative chronic myeloproliferative disorders (C-MPD) and found to have tyrosine kinase activity similar to the BCR/ABL fusion protein. We describe a case of a 61-year-old female with a C-MPD associated with an ETV6/ABL gene rearrangement. She achieved complete cytogenetic remission on imatinib 400mg daily for 17 months, but then developed morphologic and cytogenetic relapse. After starting nilotinib 400mg orally twice daily, she achieved CCyR at 3, 6, and 11 months, suggesting that second-generation TKIs can result in favorable responses in patients with ETV6/ABL rearrangement who relapse after imatinib.
authors.affiliation: Division of Neoplastic Diseases and Related Disorders, Medical College of Wisconsin, Milwaukee, WI 53226, USA. rnand@mcw.edu
authors.forename: Ranveer
authors.initials: R
authors.lastname: Nand
authors.forename: Christine
authors.initials: C
authors.lastname: Bryke
authors.forename: Steven H
authors.initials: SH
authors.lastname: Kroft
authors.forename: Ajit
authors.initials: A
authors.lastname: Divgi
authors.forename: Christopher
authors.initials: C
authors.lastname: Bredeson
authors.forename: Ehab
authors.initials: E
authors.lastname: Atallah
mesh_terms: D001549:Benzamides; D004802:Eosinophilia / Q000150:complications / Q000188:drug therapy* / Q000201:enzymology / Q000235:genetics; D005260:Female; D015321:Gene Rearrangement / Q000235:genetics; D006801:Humans; D000068877:Imatinib Mesylate; D008875:Middle Aged; D009196:Myeloproliferative Disorders / Q000150:complications / Q000188:drug therapy* / Q000201:enzymology / Q000235:genetics; D015514:Oncogene Proteins, Fusion; D010879:Piperazines / Q000008:administration & dosage*; D047428:Protein Kinase Inhibitors / Q000008:administration & dosage*; D011505:Protein-Tyrosine Kinases; D011743:Pyrimidines / Q000008:administration & dosage*; D012074:Remission Induction; D000097764:ETS Translocation Variant 6 Protein
chemical_list: D001549:Benzamides; D000068877:Imatinib Mesylate; D015514:Oncogene Proteins, Fusion; D010879:Piperazines; D047428:Protein Kinase Inhibitors; D011505:Protein-Tyrosine Kinases; D011743:Pyrimidines; C426234:TEL-ABL fusion protein, human; D000097764:ETS Translocation Variant 6 Protein