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

CS Tam, RM Nussenzveig, U Popat… - Blood, The Journal …, 2008 - ashpublications.org
CS Tam, RM Nussenzveig, U Popat, CE Bueso-Ramos, DA Thomas, JA Cortes
Blood, The Journal of the American Society of Hematology, 2008ashpublications.org
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 …
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.
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