Primary
- Congenital (erythropoietin receptor mutations)
- Polycythemia vera
- Idiopathic erythrocytosis
Secondary
- Congenital
- Defects in oxygen sensing pathway – Chuvash erythrocytosis (VHL mutation)
- Left shift of hemoglobin (Hb) oxygen dissociation curve:
- High affinity Hb
- 2,3-DPG deficiency
- Acquired
- Hypoxia-driven:
- Central process:
- Chronic lung disease
- Right-to-left cardiopulmonary shunts
- Carbon monoxide poisoning
- Smoking
- Sleep apnea
- High altitude
- Local process:
- Renal artery stenosis
- Hydronephrosis
- Renal cysts
- Central process:
- Pathological erythropoietin production:
- Tumors:
- Hepatocellular carcinoma
- Renal cell cancer
- Cerebellar hemangioblastoma
- Uterine leiomyoma
- Pheochromocytoma
- Meningioma
- Tumors:
- Drug-associated:
- Erythropoietin
- Androgens
- Diuretics
- Hypoxia-driven:
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