Table 1.
Primary erythrocytosis |
Congenital |
EPO receptor mutations |
Acquired |
Polycythemia vera |
Secondary erythrocytosis |
Congenital |
Oxygen-sensing pathway mutations |
High oxygen-affinity hemoglobin |
Bisphosphoglycerate mutase deficiency |
Acquired |
EPO mediated |
Hypoxia driven |
Central hypoxic process |
Local renal hypoxia |
Pathologic EPO production |
Tumours |
Exogenous EPO |
Drug associated |
Idiopathic erythrocytosis |
EPO, erythropoietin.