Treatment of Secondary Polycythemia in Idiopathic Erythrocytosis
This protocol covers secondary polycythemia presenting as idiopathic erythrocytosis — a diagnosis of exclusion defined by an absolute rise in red cell mass with no identifiable underlying cause.
Idiopathic erythrocytosis (IE) is a diagnosis of exclusion. It represents an absolute erythrocytosis of no identifiable cause and is more frequent in males. Individual thrombotic risk assessment is central to management decisions in this setting.
Where clinically warranted, targeted haematocrit management is the key intervention in selected patients — cytoreductive therapy is not part of the approach. Full patient-selection criteria and the complete clinical algorithm are available in the protocol.
The primary goal is reduction of haematocrit to below 0.55, or below 0.45 in patients with a history of thrombosis related to the erythrocytosis.
References
- Idiopathic erythrocytosis (IE) is a diagnosis of exclusion.
- It is an absolute erythrocytosis of no identifiable cause that is more frequent in males.
- The thrombotic risk factors should be evaluated in each case and, in selected cases, the Hct can be controlled by venesection.
- It would be reasonable to venesect patients with an arbitrary target Hct of <0.55, although a lower target Hct of <0.45 may be appropriate for a patient with a history of thrombosis considered to be related to the erythrocytosis.
- Cytoreductive therapy is not indicated in patients with IE.