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.
MeSH terms
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Benzamides
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Eosinophilia / complications
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Eosinophilia / drug therapy*
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Eosinophilia / enzymology
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Eosinophilia / genetics
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Female
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Gene Rearrangement / genetics
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Humans
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Imatinib Mesylate
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Middle Aged
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Myeloproliferative Disorders / complications
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Myeloproliferative Disorders / drug therapy*
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Myeloproliferative Disorders / enzymology
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Myeloproliferative Disorders / genetics
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Oncogene Proteins, Fusion*
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Piperazines / administration & dosage*
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Protein Kinase Inhibitors / administration & dosage*
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Protein-Tyrosine Kinases*
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Pyrimidines / administration & dosage*
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Remission Induction
Substances
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Benzamides
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Oncogene Proteins, Fusion
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Piperazines
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Protein Kinase Inhibitors
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Pyrimidines
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TEL-ABL fusion protein, human
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Imatinib Mesylate
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Protein-Tyrosine Kinases