Description | A total of 410 patients with blast phase myeloproliferative neoplasm (MPN-BP) were retrospectively reviewed: 248 from the
Mayo Clinic and 162 from Italy. Median survival was 3.6 months, with no improvement over the last 15 years. Multivariable
analysis performed on the Mayo cohort identified high risk karyotype, platelet count < 100 × 109
/L, age > 65 years and
transfusion need as independent risk factors for survival. Also in the Mayo cohort, intensive chemotherapy resulted in complete
remission (CR) or CR with incomplete count recovery (CRi) rates of 35 and 24%, respectively; treatment-specified 3-year/5-year
survival rates were 32/10% for patients receiving allogeneic stem cell transplant (AlloSCT) (n = 24), 19/13% for patients
achieving CR/CRi but were not transplanted (n = 24), and 1/1% in the absence of both AlloSCT and CR/CRi (n = 200) (p <
0.01). The survival impact of AlloSCT (HR 0.2, 95% CI 0.1–0.3), CR/CRi without AlloSCT (HR 0.3, 95% CI 0.2–0.5), high risk
karyotype (HR 1.6, 95% CI 1.1–2.2) and platelet count < 100 × 109
/L (HR 1.6, 95% CI 1.1–2.2) were confirmed to be interindependent. Similar observations were made in the Italian cohort. The current study identifies the setting for improved short-term
survival in MPN-BP, but also highlights the limited value of current therapy, including AlloSCT, in securing long-term survival. | en_US |