Table 4.

Revised IPSET-Thrombosis Model for Essential Thrombocythemia

Variables	Risk Categories	Therapy
Age ≤ 60 years old Prior thrombosis JAK2V617F mutation	Very low (Age ≤ 60 years, JAK2 wild type, no prior thrombosis)	Management of CV risk factors, observation or low dose aspirin, unless contraindicateda
	Low (Age ≤ 60 years, JAK2V617F positive, no prior thrombosis)	Management of CV risk factors and low dose aspirin unless contraindicateda. Higher dose aspirin may be used if CV risk factors present.
	Intermediate (age > 60 years, JAK2 wild type, no prior thrombosis)	Management of CV risk factors and cytoreductive therapy plus low-dose aspirin, unless contraindicateda. Higher dose aspirin without cytoreductive therapy if no CV risk factors.
	High (age > 60 years and JAK2V617F positive, or prior thrombosis)	Management of CV risk factors and cytoreductive therapy plus low-dose aspirin

Notes: ^aAspirin is contraindicated in the presence of acquired von Willebrand’s disease or active major bleedings. In bold molecular variable. Data from Barbui et al.⁵¹

Abbreviations: IPSET, International Prognostic Score for Essential Thrombocythemia; CV, cardiovascular.