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

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.

Allo-SCT for Philadelphia-negative myeloproliferative neoplasms in blast phase: a study from the Societe Française de Greffe de Moelle et de Therapie Cellulaire (SFGM-TC)

“…2,17 Similarly, transformation of chronic myelomonocytic leukemia-the most frequent MPN/MDS-into AML portends poor prognosis with a median OS of~6 months. 4 In MPN, neither intensive chemotherapy nor hypomethylating agents can allow long-term LFS unless allo-SCT is performed.…”

“…4 In MPN, neither intensive chemotherapy nor hypomethylating agents can allow long-term LFS unless allo-SCT is performed. 2,7 As blast phase occurs most of the time in elderly patients with potential comorbidities such as thrombotic events, few patients can proceed to allo-SCT. 2 Thus, the current cohort likely represents a selected group of patients who were deemed to be eligible to allo-SCT.…”

“…1 This blast phase portends a dismal prognosis with median OS not exceeding a few months. [2][3][4] Various strategies have been attempted in order to improve these patients' outcomes, ranging from intensive chemotherapy to less-intensive hypomethylating agents. However, in all cases CR rates remain o 50%.…”

“…However, in all cases CR rates remain o 50%. 2,3,5 Importantly, patients who achieve CR will ultimately relapse unless they receive allogeneic hematopoietic SCT (allo-SCT). 2,6,7 However, such reports are rare and often include small cohorts.…”

“…2,3,5 Importantly, patients who achieve CR will ultimately relapse unless they receive allogeneic hematopoietic SCT (allo-SCT). 2,6,7 However, such reports are rare and often include small cohorts. 2,[6][7][8][9][10] The aim of this study was to retrospectively investigate the outcome of patients in blast phase of an MPN-MDS/MPN who received allo-SCT.…”