TY - JOUR
T1 - Blast phase myeloproliferative neoplasm
T2 - Mayo-AGIMM study of 410 patients from two separate cohorts
AU - Tefferi, Ayalew
AU - Mudireddy, Mythri
AU - Mannelli, Francesco
AU - Begna, Kebede H.
AU - Patnaik, Mrinal M.
AU - Hanson, Curtis A.
AU - Ketterling, Rhett P.
AU - Gangat, Naseema
AU - Yogarajah, Meera
AU - De Stefano, Valerio
AU - Passamonti, Francesco
AU - Rosti, Vittorio
AU - Finazzi, Maria Chiara
AU - Rambaldi, Alessandro
AU - Bosi, Alberto
AU - Guglielmelli, Paola
AU - Pardanani, Animesh
AU - Vannucchi, Alessandro M.
N1 - Publisher Copyright:
© 2018 The Author(s).
PY - 2018/5/1
Y1 - 2018/5/1
N2 - 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 × 10 9 /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 × 10 9 /L (HR 1.6, 95% CI 1.1-2.2) were confirmed to be inter-independent. 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.
AB - 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 × 10 9 /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 × 10 9 /L (HR 1.6, 95% CI 1.1-2.2) were confirmed to be inter-independent. 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.
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U2 - 10.1038/s41375-018-0019-y
DO - 10.1038/s41375-018-0019-y
M3 - Article
C2 - 29459662
AN - SCOPUS:85042175961
SN - 0887-6924
VL - 32
SP - 1200
EP - 1210
JO - Leukemia
JF - Leukemia
IS - 5
ER -