Treatment recommendations for patients with essential thrombocythemia (ET) and polycythemia vera (PV).
| . | Essential Thrombocythemia . | Polycythemia Vera . |
|---|---|---|
| * Where possible all low and intermediate risk group patients with either ET or PV should be entered into clinical trials | ||
| ** For patients (particularly female) with on-going symptoms a further reduction of Hct to < 0.42 is recommended but not supported by published evidence | ||
| All patients: | Seek and aggressively manage all reversible risk factors for vascular disease and determine family history Aspirin 75 mg for all patients in the absence of active contraindications (for those patients with platelet count > 1000 x 109/L it is wise to exclude acquired von Willebrand disease) | |
| Low risk*: | NO cytoreductive therapy | Venesect to maintain Hct < 0.45** |
| Intermediate risk*: | NO cytoreductive therapy | Venesect to maintain Hct < 0.45** |
| High risk: | Control plt < 400 x 109/L with: Hydroxyurea (hydroxycarbamide) Consider interferon (IFN)α or anagrelide for young patients (< 40 years) | Control Hct < 0.45 and plt < 400 x 109/L with: Hydroxyurea (hydroxycarbamide) Consider IFNα or anagrelide for young patients (< 40 years) |
| Refractory or Intolerant: | Change cytotoxic to one of low leukemogenic potential, i.e., consider IFNα or anagrelide patients > 75 years busulfan or P32 | Change cytotoxic to one of low leukemogenic potential, i.e., consider IFNα or anagrelide patients > 75 years busulfan or P32 |
| . | Essential Thrombocythemia . | Polycythemia Vera . |
|---|---|---|
| * Where possible all low and intermediate risk group patients with either ET or PV should be entered into clinical trials | ||
| ** For patients (particularly female) with on-going symptoms a further reduction of Hct to < 0.42 is recommended but not supported by published evidence | ||
| All patients: | Seek and aggressively manage all reversible risk factors for vascular disease and determine family history Aspirin 75 mg for all patients in the absence of active contraindications (for those patients with platelet count > 1000 x 109/L it is wise to exclude acquired von Willebrand disease) | |
| Low risk*: | NO cytoreductive therapy | Venesect to maintain Hct < 0.45** |
| Intermediate risk*: | NO cytoreductive therapy | Venesect to maintain Hct < 0.45** |
| High risk: | Control plt < 400 x 109/L with: Hydroxyurea (hydroxycarbamide) Consider interferon (IFN)α or anagrelide for young patients (< 40 years) | Control Hct < 0.45 and plt < 400 x 109/L with: Hydroxyurea (hydroxycarbamide) Consider IFNα or anagrelide for young patients (< 40 years) |
| Refractory or Intolerant: | Change cytotoxic to one of low leukemogenic potential, i.e., consider IFNα or anagrelide patients > 75 years busulfan or P32 | Change cytotoxic to one of low leukemogenic potential, i.e., consider IFNα or anagrelide patients > 75 years busulfan or P32 |