Table 1.

Patient, disease, and treatment characteristics associated with risk of progression to MPN-AP/MPN-BP

Patient factor 
 Advanced patient age (>60 vs >70 years)57,58  
Disease factors 
 Longer disease duration31  
 Higher risk for patients with PMF compared to ET and PV11  
 Platelet count <150 × 109/L, leukocytosis, transfusion dependence, peripheral blasts ≥3%16,58,59  
 Splenomegaly of ≥20 cm from left costal margin or requirement for splenectomy59  
 Bone marrow reticulin fibrosis57  
 Abnormal karyotype58,59  
 Lower risk with CALR mutations60  
 High-risk mutations (eg, TP53, ASXL1, SRSF2, IDH2, EZH2)4,60  
 Higher DIPSS, MYSEC-PM, or MIPSS70v2.0 score16  
Treatment factors 
 Prior treatment with alkylating agents and pipobroman20,58  
 Lower risk in patients treated with IFN16  
Patient factor 
 Advanced patient age (>60 vs >70 years)57,58  
Disease factors 
 Longer disease duration31  
 Higher risk for patients with PMF compared to ET and PV11  
 Platelet count <150 × 109/L, leukocytosis, transfusion dependence, peripheral blasts ≥3%16,58,59  
 Splenomegaly of ≥20 cm from left costal margin or requirement for splenectomy59  
 Bone marrow reticulin fibrosis57  
 Abnormal karyotype58,59  
 Lower risk with CALR mutations60  
 High-risk mutations (eg, TP53, ASXL1, SRSF2, IDH2, EZH2)4,60  
 Higher DIPSS, MYSEC-PM, or MIPSS70v2.0 score16  
Treatment factors 
 Prior treatment with alkylating agents and pipobroman20,58  
 Lower risk in patients treated with IFN16  

DIPSS, Dynamic International Prognostic Scoring System; MYSEC-PM, myelofibrosis secondary to PV and ET Collaboration Prognostic Model.

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