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

@article{citeulike:3753817, 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 JAK2V617F\% 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. 10.1182/blood-2008-02-138230}, author = {Tam, Constantine S. and Nussenzveig, Roberto M. and Popat, Uday and Bueso-Ramos, Carlos E. and Thomas, Deborah A. and Cortes, Jorge A. and Champlin, Richard E. and Ciurea, Stefan E. and Manshouri, Taghi and Pierce, Sherry M. and Kantarjian, Hagop M. and Verstovsek, Srdan}, citeulike-article-id = {3753817}, citeulike-linkout-0 = {http://dx.doi.org/10.1182/blood-2008-02-138230}, citeulike-linkout-1 = {http://bloodjournal.hematologylibrary.org/cgi/content/abstract/112/5/1628}, citeulike-linkout-2 = {http://view.ncbi.nlm.nih.gov/pubmed/18566326}, citeulike-linkout-3 = {http://www.hubmed.org/display.cgi?uids=18566326}, day = {1}, doi = {10.1182/blood-2008-02-138230}, journal = {Blood}, keywords = {mpd}, month = {September}, number = {5}, pages = {1628--1637}, posted-at = {2008-12-07 23:31:59}, priority = {2}, title = {The natural history and treatment outcome of blast phase BCR-ABL- myeloproliferative neoplasms}, url = {http://dx.doi.org/10.1182/blood-2008-02-138230}, volume = {112}, year = {2008} }

TY - JOUR ID - citeulike:3753817 L3 - citeulike-article-id:3753817 N2 - 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 JAK2V617F% 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. 10.1182/blood-2008-02-138230 IS - 5 JF - Blood EP - 1637 TI - The natural history and treatment outcome of blast phase BCR-ABL- myeloproliferative neoplasms VL - 112 SP - 1628 KW - mpd AU - Tam, Constantine AU - Nussenzveig, Roberto AU - Popat, Uday AU - Bueso-Ramos, Carlos AU - Thomas, Deborah AU - Cortes, Jorge AU - Champlin, Richard AU - Ciurea, Stefan AU - Manshouri, Taghi AU - Pierce, Sherry AU - Kantarjian, Hagop AU - Verstovsek, Srdan PY - 2008/09/01/ UR - http://dx.doi.org/10.1182/blood-2008-02-138230 ER -