Polycythemia classification: Difference between revisions
Jump to navigation
Jump to search
| Line 4: | Line 4: | ||
==Overview== | ==Overview== | ||
Mainly classified into primary and secondary causes based on the presence or absence of genetic mutations and underlying disorders. | 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:<ref name="McMULLIN2008">{{cite journal|last1=McMULLIN|first1=M. F.|title=The classification and diagnosis of erythrocytosis|journal=International Journal of Laboratory Hematology|year=2008|issn=17515521|doi=10.1111/j.1751-553X.2008.01102.x}}</ref> | Once the diagnosis of absolute [[erythrocytosis]] has been established, red cell mass (RCM > 125% of predicted), classification can be done accordingly:<ref name="McMULLIN2008">{{cite journal|last1=McMULLIN|first1=M. F.|title=The classification and diagnosis of erythrocytosis|journal=International Journal of Laboratory Hematology|year=2008|issn=17515521|doi=10.1111/j.1751-553X.2008.01102.x}}</ref> | ||
*Primary erythrocytosis | * '''Primary [[erythrocytosis]]''' | ||
- Polycythemia vera | - [[Polycythemia vera]] | ||
*Secondary erythrocytosis | *'''Secondary [[erythrocytosis]]''' | ||
Congenital: | [[Congenital]]: | ||
- Erythropoietin receptor-mediated | - Erythropoietin receptor-mediated | ||
- High oxygen affinity hemoglobin | - High oxygen affinity hemoglobin | ||
| Line 23: | Line 23: | ||
- PHD2 mutations | - PHD2 mutations | ||
- HIF-2 alpha mutations | - HIF-2 alpha mutations | ||
Acquired: | [[Acquired]]: | ||
- Hypoxia driven | - Hypoxia driven | ||
- Central hypoxic process: | - Central hypoxic process: | ||
| Line 50: | Line 50: | ||
- EPO administration | - EPO administration | ||
- Androgen administration | - Androgen administration | ||
Idiopathic erythrocytosis <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> | [[Idiopathic]] erythrocytosis <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== | ||
Revision as of 17:10, 25 February 2021
|
Polycythemia Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Polycythemia classification On the Web |
|
American Roentgen Ray Society Images of Polycythemia classification |
|
Risk calculators and risk factors for Polycythemia classification |
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:[1]
- Primary erythrocytosis
- Secondary erythrocytosis
- Erythropoietin receptor-mediated
- High oxygen affinity hemoglobin
- Bisphosphoglycerate mutase deficiency
- VHL (Von Hippel-Lindau) gene mutation (Chuvash erythrocytosis)
- PHD2 mutations
- HIF-2 alpha mutations
- 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 [2]
References
- ↑ McMULLIN, M. F. (2008). "The classification and diagnosis of erythrocytosis". International Journal of Laboratory Hematology. doi:10.1111/j.1751-553X.2008.01102.x. ISSN 1751-5521.
- ↑ 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.