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. 2018 May;32(5):1200-1210.
doi: 10.1038/s41375-018-0019-y. Epub 2018 Feb 2.

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

Ayalew Tefferi  1 , Mythri Mudireddy  2 , Francesco Mannelli  3   4 , Kebede H Begna  2 , Mrinal M Patnaik  2 , Curtis A Hanson  2 , Rhett P Ketterling  2 , Naseema Gangat  2 , Meera Yogarajah  5 , Valerio De Stefano  3   6 , Francesco Passamonti  3   7 , Vittorio Rosti  3   8 , Maria Chiara Finazzi  3   9 , Alessandro Rambaldi  3   9 , Alberto Bosi  3   4 , Paola Guglielmelli  3   4 , Animesh Pardanani  2 , Alessandro M Vannucchi  3   4
Affiliations

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

Ayalew Tefferi et al. Leukemia. 2018 May.

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.

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Conflict of interest statement

The authors declare that they have no conflict of interest.

Figures

Fig. 1
Fig. 1
a Survival data on 248 Mayo Clinic patients with blast-phase myeloproliferative neoplasm, stratified by year of diagnosis; b Survival data on 162 Italian patients with blast-phase myeloproliferative neoplasm, stratified by year of diagnosis
Fig. 2
Fig. 2
a Survival data on 248 Mayo Clinic patients with blast-phase myeloproliferative neoplasm, stratified by specific treatment strategies; b Survival data on 162 Italian patients with blast-phase myeloproliferative neoplasm, stratified by treatment received
Fig. 3
Fig. 3
a Survival data on 66 Mayo Clinic patients with blast-phase myeloproliferative neoplasm, treated with intensive induction chemotherapy and stratified by specific response categories; b Survival data on 24 Mayo Clinic patients with blast-phase myeloproliferative neoplasm, treated with allogeneic stem cell transplantation and stratified by specific response category at time of transplant; c Survival data on 21 Mayo Clinic patients with blast-phase myeloproliferative neoplasm who were transplanted and stratified by the presence or absence of high risk karyotype
See this image and copyright information in PMC

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