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Blood, 1 September 2008, Vol. 112, No. 5, pp. 1628-1637. Prepublished online as a Blood First Edition Paper on June 19, 2008; DOI 10.1182/blood-2008-02-138230. This Article Full Text (PDF) All Versions of this Article: blood-2008-02-138230v1 112/5/1628    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Tam, C. S Articles by Verstovsek, S. Search for Related Content PubMed PubMed Citation Articles by Tam, C. S Articles by Verstovsek, S. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted February 20, 2008 Accepted May 11, 2008 The natural history and treatment outcome of blast phase BCR-ABL negative 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, and Srdan Verstovsek* Leukemia, University of Texas MD Anderson Cancer Center, Houston, Texas Stem Cell Transplantation & Cellular Therapy, University of Texas MD Anderson Cancer Center, Houston, Texas Hematopathology, University of Texas MD Anderson Cancer Center, Houston, Texas * Corresponding author; email: sverstov{at}mdanderson.org. We analyzed the outcomes of 74 patients diagnosed with BCR-ABL negative myeloproliferative neoplasms (MPN) 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 ± blood recovery was achieved in 46% of patients receiving induction chemotherapy, but remissions were not durable with a median PFS of only 5 months. Eight patients received SCT either as first therapy or after responding to anti-leukemia therapy. These patients have a markedly superior survival, with 73% being 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 JAK2V617F% at transformation, 7 had a substantial decrease, and 4 had stable levels. MPN 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. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: C. S. Tam, H. Kantarjian, J. Cortes, A. Lynn, S. Pierce, L. Zhou, M. J. Keating, D. A. Thomas, and S. Verstovsek Dynamic Model for Predicting Death Within 12 Months in Patients With Primary or Post-Polycythemia Vera/Essential Thrombocythemia Myelofibrosis J. Clin. Oncol., November 20, 2009; 27(33): 5587 - 5593. [Abstract] [Full Text] [PDF] A. M. Vannucchi, P. Guglielmelli, and A. Tefferi Advances in Understanding and Management of Myeloproliferative Neoplasms CA Cancer J Clin, May 1, 2009; 59(3): 171 - 191. [Abstract] [Full Text] [PDF]

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