Table 1.

Risk factors for myeloproliferative neoplasm bleeding

Risk factorComment
Advanced age Prospective PV cohort,68  retrospective study of all MPN subtypes67  
Disease duration Prospective PV cohort68  
Splenomegaly Odds ratio 2.2 in German prospective cohort 
Prior thrombosis Odds ratio 2.7 in German prospective cohort 
Portal HTN As in BCS and PVT, or with massive splenomegaly or hepatic extramedullary hematopoiesis 
Prior hemorrhage WHO-defined ET and PMF, as well as PV68  
MPN subtype MF > ET/PV 
Driving mutation JAK2 V617F may associate with aVWS71  in ET 
Unclear if risk is greater in CALR vs JAK2 V617F-positive MPNs 
Thrombocytosis With or without aVWS 
Leukocytosis ET and early MF 
aVWS Identified even in absence of extreme thrombocytosis71  
Treatment Antiplatelet therapy? 
Anticoagulation? 
Antiplatelet therapy with anagrelide41  
Risk factorComment
Advanced age Prospective PV cohort,68  retrospective study of all MPN subtypes67  
Disease duration Prospective PV cohort68  
Splenomegaly Odds ratio 2.2 in German prospective cohort 
Prior thrombosis Odds ratio 2.7 in German prospective cohort 
Portal HTN As in BCS and PVT, or with massive splenomegaly or hepatic extramedullary hematopoiesis 
Prior hemorrhage WHO-defined ET and PMF, as well as PV68  
MPN subtype MF > ET/PV 
Driving mutation JAK2 V617F may associate with aVWS71  in ET 
Unclear if risk is greater in CALR vs JAK2 V617F-positive MPNs 
Thrombocytosis With or without aVWS 
Leukocytosis ET and early MF 
aVWS Identified even in absence of extreme thrombocytosis71  
Treatment Antiplatelet therapy? 
Anticoagulation? 
Antiplatelet therapy with anagrelide41  

aVWS, acquired von Willebrand syndrome; BCS, Budd-Chiari syndrome; ET, essential thrombocythemia; HTN, hypertension; MF, myelofibrosis; MPN, myeloproliferative neoplasm; PMF, primary myelofibrosis; PV, polycythemia vera; PVT, portal vein thrombosis; WHO, World Health Organization.

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