Table 3.
Risk stratification and preferred treatment options for essential thrombocytosis (adapted from [6])
| History of thrombosis | Age > 60 years | JAK2 mutation | CV-RF− | CV-RF+ | |
|---|---|---|---|---|---|
| Very low-risk disease | No | No | No | None | ASA |
| Low-risk disease | No | No | Yes | ASA q.d.—b.i. d. | ASA b.i. d. |
| Intermediate-risk disease | No | Yes | No | ASA (± HU) | HU + ASA |
| High-risk disease | No | Yes | Yes | HU + ASA b.i. d. | HU + ASA b.i. d. |
| High-risk disease | Yes, arterial | No (or yes) | No (or yes) | HU + ASA b.i. d. | HU + ASA b.i. d. |
| High-risk disease | Yes, venous | No (or yes) | No | HU + s-ac | HU + s-ac + ASA |
| High-risk disease | Yes, venous | No (or yes) | Yes | HU + s-ac + ASA b.i. d. | HU + s-ac + ASA b.i. d. |
NB Treatment with cytoreductive therapy should be avoided in the presence of extreme thrombocytosis (platelets > 1500 × 109/l) and acquired von Willebrand syndrome
CV-RF cardiovascular risk factors − (absent), + (present), ASA acetylsalicylic acid, HU hydroxyurea, s‑ac Systemic anticoagulation, b.i.d. twice a day