The natural history and treatment outcome of blast phase BCR-ABL - myeloproliferative neoplasms

Constantine S. Tam, Roberto M. Nussenzveig, Uday Popat, Carlos E. Bueso-Ramos, Deborah A. Thomas, Jorge A. Cortes, Richard E. Champlin, Stefan E. Ciurea, Taghi Manshouri, Sherry M. Pierce, Hagop M. Kantarjian, Srdan Verstovsek

Research output: Contribution to journalArticle

105 Scopus citations

Abstract

We analyzed the outcomes of 74 patients diagnosed with BCR-ABL - myeloproliferative neoplasms in blast phase receiving induction chemotherapy (55%), low-intensity therapy (16%), stem cell transplantation (SCT; 3%), or supportive care (26%). Median survival from the date of blastic transformation was 5 months. Patients receiving supportive therapy had a median survival of 6 weeks. Complete remission with or without blood recovery was achieved in 46% of patients receiving induction chemotherapy, but remissions were not durable with a median progression-free survival of only 5 months. Eight patients received SCT either as first therapy or after responding to antileukemia therapy. These patients had a markedly superior survival, with 73% alive at a median follow-up of 31 months. JAK2V617F kinetics were assessed in 16 patients: 0 of 4 negative patients became positive at transformation, and among 12 positive patients, 1 had an increase in J4K2V617F% at transformation, 7 had a substantial decrease, and 4 had stable levels. Myeloproliferative neoplasm blast phase is associated with a dismal prognosis. Responses to chemotherapy can be achieved but are not durable. Long-term survivors had all received SCT either as first therapy or in first remission,

Original languageEnglish (US)
Pages (from-to)1628-1637
Number of pages10
JournalBlood
Volume112
Issue number5
DOIs
StatePublished - Sep 1 2008
Externally publishedYes

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

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    Tam, C. S., Nussenzveig, R. M., Popat, U., Bueso-Ramos, C. E., Thomas, D. A., Cortes, J. A., Champlin, R. E., Ciurea, S. E., Manshouri, T., Pierce, S. M., Kantarjian, H. M., & Verstovsek, S. (2008). The natural history and treatment outcome of blast phase BCR-ABL - myeloproliferative neoplasms. Blood, 112(5), 1628-1637. https://doi.org/10.1182/blood-2008-02-138230