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 journalArticlepeer-review

139 Scopus citations


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
Issue number5
StatePublished - Sep 1 2008
Externally publishedYes

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology


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