Table 2.

Selected studies of treatment outcomes in MPN-BP

Therapeutic regimenPatient populationStudy designKey outcomesReference
Intensive chemotherapy 
 Intensive chemotherapy (not specified) MPN-BP Retrospective cohort study Median OS 10.2 mo 61  
 “7 + 3” cytarabine plus an anthracycline MPN-BP Retrospective cohort study CR rate 67%; median OS 19.2 mo for allo-HCT vs 3.8 mo for nontransplant patients  
 AML-like induction chemotherapy MPN-BP Retrospective cohort study CR rate 35%; 3-y OS 32% for allo-HCT vs 19% for nontransplant patients  
HMA monotherapy 
 Azacitidine MPN-BP/AP Retrospective cohort study ORR 61.5%; median OS 13.5 mo 43  
MPN-BP Retrospective cohort study ORR 68%; median OS 9.9 mo 42  
MPN-BP Retrospective cohort study Median OS 9 mo 61  
 Azacitidine or decitabine MPN-BP Retrospective cohort study Median OS 6.7 mo  
 Decitabine MPN-BP, MPN-AP, high-risk PMF Retrospective cohort study MPN-BP: 29% ORR, 10.5 mo median OS
MPN-AP: 62% ORR, 11.8 mo median OS 
41  
Ruxolitinib monotherapy 
 Ruxolitinib MPN-BP Phase 2 clinical trial ORR 16.6% 48  
HMA + ruxolitinib 
 Ruxolitinib + decitabine MPN-AP, MPN-BP Phase 2 clinical trial ORR 44%; median OS 9.5 mo 49  
MPN-BP Phase 1/2 clinical trial ORR 45%; median OS 8.4 mo 50  
HMA + venetoclax 
 HMA + venetoclax MPN-BP Retrospective cohort study Newly diagnosed: ORR 50%; median OS 7 mo
R/R: no objective responses; median OS 3 mo 
45  
MPN-BP Retrospective cohort study ORR 44%; median OS 8 mo 44  
 MPN-AP, MPN-BP Retrospective multicenter cohort study ORR: 52%; median OS 6 mo 47  
IDH1/2 inhibitors 
 Enasidenib IDH2-mutant MPN-AP, MPN-BP Retrospective cohort study ORR: 37.5% per ELN 2017 and 75% per MPN-BP response criteria 54  
 Enasidenib or ivosidenib IDH1 or IDH2-mutant MPN-AP, MPN-BP Retrospective cohort study 43% CR rate among newly diagnosed patients; no objective responses in R/R cohort 53  
Therapeutic regimenPatient populationStudy designKey outcomesReference
Intensive chemotherapy 
 Intensive chemotherapy (not specified) MPN-BP Retrospective cohort study Median OS 10.2 mo 61  
 “7 + 3” cytarabine plus an anthracycline MPN-BP Retrospective cohort study CR rate 67%; median OS 19.2 mo for allo-HCT vs 3.8 mo for nontransplant patients  
 AML-like induction chemotherapy MPN-BP Retrospective cohort study CR rate 35%; 3-y OS 32% for allo-HCT vs 19% for nontransplant patients  
HMA monotherapy 
 Azacitidine MPN-BP/AP Retrospective cohort study ORR 61.5%; median OS 13.5 mo 43  
MPN-BP Retrospective cohort study ORR 68%; median OS 9.9 mo 42  
MPN-BP Retrospective cohort study Median OS 9 mo 61  
 Azacitidine or decitabine MPN-BP Retrospective cohort study Median OS 6.7 mo  
 Decitabine MPN-BP, MPN-AP, high-risk PMF Retrospective cohort study MPN-BP: 29% ORR, 10.5 mo median OS
MPN-AP: 62% ORR, 11.8 mo median OS 
41  
Ruxolitinib monotherapy 
 Ruxolitinib MPN-BP Phase 2 clinical trial ORR 16.6% 48  
HMA + ruxolitinib 
 Ruxolitinib + decitabine MPN-AP, MPN-BP Phase 2 clinical trial ORR 44%; median OS 9.5 mo 49  
MPN-BP Phase 1/2 clinical trial ORR 45%; median OS 8.4 mo 50  
HMA + venetoclax 
 HMA + venetoclax MPN-BP Retrospective cohort study Newly diagnosed: ORR 50%; median OS 7 mo
R/R: no objective responses; median OS 3 mo 
45  
MPN-BP Retrospective cohort study ORR 44%; median OS 8 mo 44  
 MPN-AP, MPN-BP Retrospective multicenter cohort study ORR: 52%; median OS 6 mo 47  
IDH1/2 inhibitors 
 Enasidenib IDH2-mutant MPN-AP, MPN-BP Retrospective cohort study ORR: 37.5% per ELN 2017 and 75% per MPN-BP response criteria 54  
 Enasidenib or ivosidenib IDH1 or IDH2-mutant MPN-AP, MPN-BP Retrospective cohort study 43% CR rate among newly diagnosed patients; no objective responses in R/R cohort 53  

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