Blast phase myeloproliferative neoplasm: Mayo-AGIMM study of 410 patients from two separate cohorts

Ayalew Tefferi, Mythri Mudireddy, Francesco Mannelli, Kebede Begna, Mrinal M Patnaik, Curtis A. Hanson, Rhett P. Ketterling, Naseema Gangat, Meera Yogarajah, Valerio de Stefano, Francesco Passamonti, Vittorio Rosti, Maria Chiara Finazzi, Alessandro Rambaldi, Alberto Bosi, Paola Guglielmelli, Animesh D Pardanani, Alessandro M. Vannucchi

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17 Scopus citations

Abstract

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 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.

Original languageEnglish (US)
Pages (from-to)1-11
Number of pages11
JournalLeukemia
DOIs
StateAccepted/In press - Feb 2 2018

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ASJC Scopus subject areas

  • Hematology
  • Cancer Research
  • Anesthesiology and Pain Medicine

Cite this

Tefferi, A., Mudireddy, M., Mannelli, F., Begna, K., Patnaik, M. M., Hanson, C. A., Ketterling, R. P., Gangat, N., Yogarajah, M., de Stefano, V., Passamonti, F., Rosti, V., Finazzi, M. C., Rambaldi, A., Bosi, A., Guglielmelli, P., Pardanani, A. D., & Vannucchi, A. M. (Accepted/In press). Blast phase myeloproliferative neoplasm: Mayo-AGIMM study of 410 patients from two separate cohorts. Leukemia, 1-11. https://doi.org/10.1038/s41375-018-0019-y