Table 1.

Risk stratification for thrombosis in patients with essential thrombocythemia (ET) and polycythemia vera (PV).

Essential ThrombocythemiaPolycythemia Vera
* The role of familial thrombophilia or cardiovascular disease in risk stratification is controversial. 
** An intermediate risk catergory particularly for PV is controversial 
Low risk: Patients aged less than 40 years with all of the following:
 NO prior thrombosis or hemorrhage
 NO hypertension or diabetes
 Platelet count < 1500 x 109/L
 NO familial thrombophilia/cardiovascular disease* Patients aged less than 40 years with all of the following:
 NO prior thrombosis or hemorrhage
 NO congestive cardiac failure, hypertension or diabetes
 NO familial thrombophilia/cardiovascular disease*
 NO symptomatic or progressive splenomegaly 
Intermediate risk: **Patients aged 40–60 years with all of the following:
 NO prior thrombosis
 NO hypertension or diabetes
 Platelet count < 1500 x 109/L
 OR
 Patients aged < 60 years and: familial thrombophilia/cardiovascular disease* Patients aged 40–60 years with all of the following
 NO prior thrombosis
 NO congestive cardiac failure, hypertension or diabetes
 Platelet count < 1500 x 109/L
 NO progressive or symptomatic splenomegaly
 OR
 Patients aged < 60 years and: familial thrombophilia/cardiovascular disease* 
High risk: Patients either age > 60 years, or with any one of the following:
 Prior thrombosis or hemorrhage
 Platelet count > 1000 – 1500 x 109/)
 Hypertension
 Diabetes requiring treatment Patients either age > 60 years, or with any one of the following:
 Prior thrombosis or hemorrhage
 Platelet count > 1000–1500 x 109/L)
 Congestive cardiac failure
 Hypertension
 Diabetes
 Progressive or symptomatic splenomegaly 
Essential ThrombocythemiaPolycythemia Vera
* The role of familial thrombophilia or cardiovascular disease in risk stratification is controversial. 
** An intermediate risk catergory particularly for PV is controversial 
Low risk: Patients aged less than 40 years with all of the following:
 NO prior thrombosis or hemorrhage
 NO hypertension or diabetes
 Platelet count < 1500 x 109/L
 NO familial thrombophilia/cardiovascular disease* Patients aged less than 40 years with all of the following:
 NO prior thrombosis or hemorrhage
 NO congestive cardiac failure, hypertension or diabetes
 NO familial thrombophilia/cardiovascular disease*
 NO symptomatic or progressive splenomegaly 
Intermediate risk: **Patients aged 40–60 years with all of the following:
 NO prior thrombosis
 NO hypertension or diabetes
 Platelet count < 1500 x 109/L
 OR
 Patients aged < 60 years and: familial thrombophilia/cardiovascular disease* Patients aged 40–60 years with all of the following
 NO prior thrombosis
 NO congestive cardiac failure, hypertension or diabetes
 Platelet count < 1500 x 109/L
 NO progressive or symptomatic splenomegaly
 OR
 Patients aged < 60 years and: familial thrombophilia/cardiovascular disease* 
High risk: Patients either age > 60 years, or with any one of the following:
 Prior thrombosis or hemorrhage
 Platelet count > 1000 – 1500 x 109/)
 Hypertension
 Diabetes requiring treatment Patients either age > 60 years, or with any one of the following:
 Prior thrombosis or hemorrhage
 Platelet count > 1000–1500 x 109/L)
 Congestive cardiac failure
 Hypertension
 Diabetes
 Progressive or symptomatic splenomegaly 
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