Polycythemia classification: Difference between revisions
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==Overview== | ==Overview== | ||
Mainly classified into primary and secondary causes based on the presence or absence of genetic mutations and underlying disorders. | |||
==Classification== | ==Classification== | ||
Once the diagnosis of absolute erythrocytosis has been established, red cell mass (RCM > 125% of predicted), classification can be done accordingly : | Once the diagnosis of absolute erythrocytosis has been established, red cell mass (RCM > 125% of predicted), classification can be done accordingly: | ||
*Primary erythrocytosis | *Primary erythrocytosis | ||
- Polycythemia vera | - Polycythemia vera | ||
*Secondary erythrocytosis | *Secondary erythrocytosis | ||
Congenital: | |||
- Erythropoietin receptor-mediated | - Erythropoietin receptor-mediated | ||
- High oxygen affinity hemoglobin | - High oxygen affinity hemoglobin | ||
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- PHD2 mutations | - PHD2 mutations | ||
- HIF-2 alpha mutations | - HIF-2 alpha mutations | ||
Acquired: | |||
- Hypoxia driven | - Hypoxia driven | ||
- Central hypoxic process: | - Central hypoxic process: | ||
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- EPO administration | - EPO administration | ||
- Androgen administration | - Androgen administration | ||
Idiopathic erythrocytosis <ref name="urlThe classification and diagnosis of erythrocytosis - McMULLIN - 2008 - International Journal of Laboratory Hematology - Wiley Online Library">{{cite web |url=https://onlinelibrary.wiley.com/doi/full/10.1111/j.1751-553X.2008.01102.x |title=The classification and diagnosis of erythrocytosis - McMULLIN - 2008 - International Journal of Laboratory Hematology - Wiley Online Library |format= |work= |accessdate=}}</ref> <ref name="pmid967201">{{cite journal |vauthors=Adamson JW, Fialkow PJ, Murphy S, Prchal JF, Steinmann L |title=Polycythemia vera: stem-cell and probable clonal origin of the disease |journal=N Engl J Med |volume=295 |issue=17 |pages=913–6 |date=October 1976 |pmid=967201 |doi=10.1056/NEJM197610212951702 |url=}}</ref> | |||
==References== | ==References== | ||
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[[Category:Disease]] | [[Category:Disease]] | ||
[[Category:Blood disorders]] | [[Category:Blood disorders]] | ||
Revision as of 01:27, 22 February 2021
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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.