TY - JOUR
T1 - The natural history and treatment outcome of blast phase BCR-ABL - myeloproliferative neoplasms
AU - Tam, Constantine S.
AU - Nussenzveig, Roberto M.
AU - Popat, Uday
AU - Bueso-Ramos, Carlos E.
AU - Thomas, Deborah A.
AU - Cortes, Jorge A.
AU - Champlin, Richard E.
AU - Ciurea, Stefan E.
AU - Manshouri, Taghi
AU - Pierce, Sherry M.
AU - Kantarjian, Hagop M.
AU - Verstovsek, Srdan
PY - 2008/9/1
Y1 - 2008/9/1
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 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,
AB - 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,
UR - http://www.scopus.com/inward/record.url?scp=52649131772&partnerID=8YFLogxK
U2 - 10.1182/blood-2008-02-138230
DO - 10.1182/blood-2008-02-138230
M3 - Article
C2 - 18566326
AN - SCOPUS:52649131772
SN - 0006-4971
VL - 112
SP - 1628
EP - 1637
JO - Blood
JF - Blood
IS - 5
ER -