Polycythemia classification
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Debduti Mukhopadhyay, M.B.B.S[2]
Overview
- Mainly classified into primary and secondary causes based on the presence or absence of genetic mutations and underlying disorders.
Classification
Once the diagnosis of absolute erythrocytosis has been established, red cell mass (RCM > 125% of predicted), classification can be done accordingly :
- Primary erythrocytosis
- Polycythemia vera
- Secondary erythrocytosis
- Congenital:
- Erythropoietin receptor-mediated
- High oxygen affinity hemoglobin
- Bisphosphoglycerate mutase deficiency
- VHL (Von Hippel-Lindau) gene mutation (Chuvash erythrocytosis)
- PHD2 mutations
- HIF-2 alpha mutations
- Acquired:
- Hypoxia driven
- Central hypoxic process:
- Chronic Lung disease
- Right-to-left cardiopulmonary vascular shunts
- Carbon monoxide poisoning
- Smoker's erythrocytosis
- Hypoventilation syndromes including sleep apnea (high-altitude habitat)
- Local renal hypoxia:
- Renal Artery Stenosis
- End Stage Renal Disease
- Hydronephrosis
- Renal cysts (polycystic kidney disease)
- Postrenal transplant erythrocytosis
- Pathologic EPO production:
- Tumors
- Cerebellar hemangioblastoma
- Meningioma
- Parathyroid carcinoma/adenomas
- Hepatocellular carcinoma
- Renal cell cancer
- Pheochromocytoma
- Uterine leiomyomas
- Exogenous EPO:
- Drug associated
- EPO administration
- Androgen administration
- Idiopathic erythrocytosis [1] [2]
References
- ↑ "The classification and diagnosis of erythrocytosis - McMULLIN - 2008 - International Journal of Laboratory Hematology - Wiley Online Library".
- ↑ Adamson JW, Fialkow PJ, Murphy S, Prchal JF, Steinmann L (October 1976). "Polycythemia vera: stem-cell and probable clonal origin of the disease". N Engl J Med. 295 (17): 913–6. doi:10.1056/NEJM197610212951702. PMID 967201.