Polycythemia classification: Difference between revisions
Jump to navigation
Jump to search
Homa Najafi (talk | contribs) No edit summary |
|||
(21 intermediate revisions by 4 users not shown) | |||
Line 1: | Line 1: | ||
__NOTOC__ | __NOTOC__ | ||
{{Polycythemia}} | {{Polycythemia}} | ||
{{CMG}} | {{CMG}}{{AE}}{{Debduti}} | ||
==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:<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><ref name="pmid10640213">{{cite journal| author=Pearson TC| title=Diagnosis and classification of erythrocytoses and thrombocytoses. | journal=Baillieres Clin Haematol | year= 1998 | volume= 11 | issue= 4 | pages= 695-720 | pmid=10640213 | doi=10.1016/s0950-3536(98)80035-8 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10640213 }}</ref><ref name="pmid7018337">{{cite journal| author=Golde DW, Hocking WG, Koeffler HP, Adamson JW| title=Polycythemia: mechanisms and management. | journal=Ann Intern Med | year= 1981 | volume= 95 | issue= 1 | pages= 71-87 | pmid=7018337 | doi=10.7326/0003-4819-95-1-71 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7018337 }}</ref><ref name="ErslevCaro2009">{{cite journal|last1=Erslev|first1=A. J.|last2=Caro|first2=J.|title=Pathophysiology and Classification of Polycythaemia|journal=Scandinavian Journal of Haematology|volume=31|issue=4|year=2009|pages=287–292|issn=0036553X|doi=10.1111/j.1600-0609.1983.tb00655.x}}</ref> | |||
[ | |||
*'''Primary [[erythrocytosis]]'''<ref name="pmid10646073">{{cite journal| author=Messinezy M, Pearson TC| title=The classification and diagnostic criteria of the erythrocytoses (polycythaemias) | journal=Clin Lab Haematol | year= 1999 | volume= 21 | issue= 5 | pages= 309-16 | pmid=10646073 | doi=10.1046/j.1365-2257.1999.00246.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10646073 }}</ref><ref name="pmid18823397">{{cite journal| author=McMullin MF| title=The classification and diagnosis of erythrocytosis. | journal=Int J Lab Hematol | year= 2008 | volume= 30 | issue= 6 | pages= 447-59 | pmid=18823397 | doi=10.1111/j.1751-553X.2008.01102.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18823397 }}</ref> | |||
- [[Polycythemia vera]] | |||
*'''Secondary [[erythrocytosis]]'''<ref name="pmid14560779">{{cite journal| author=Prchal JT| title=Classification and molecular biology of polycythemias (erythrocytoses) and thrombocytosis. | journal=Hematol Oncol Clin North Am | year= 2003 | volume= 17 | issue= 5 | pages= 1151-8, vi | pmid=14560779 | doi=10.1016/s0889-8588(03)00090-x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14560779 }}</ref> | |||
[[Congenital]]: | |||
- [[Erythropoietin receptor]]-mediated | |||
- High oxygen affinity [[hemoglobin]] | |||
- [[Bisphosphoglycerate mutase|Bisphosphoglycerate]] mutase deficiency | |||
- [[VHL]] ([[Von Hippel-Lindau Disease|Von Hippel-Lindau]]) [[gene mutation]] (Chuvash [[erythrocytosis]]) | |||
- PHD2 ([[prolyl hydroxylase]] domain) 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|sleep apne]]<nowiki/>a (high-altitude habitat) | |||
- Local [[renal]] [[hypoxia]]: | |||
- [[Renal artery stenosis|Renal Artery Stenosis]] | |||
- End Stage Renal Disease | |||
- [[Hydronephrosis]] | |||
- [[Renal cyst|Renal cysts]] ([[polycystic kidney disease]]) | |||
- Postrenal [[transplant]] [[Polycythemia|erythrocytosis]] | |||
- Pathologic EPO production: | |||
- [[Tumors]] | |||
- [[Cerebellum|Cerebellar]] [[hemangioblastoma]] | |||
- [[Meningioma]] | |||
- [[Parathyroid]] [[carcinoma]]/[[Adenoma|adenomas]] | |||
- [[Hepatocellular carcinoma]] | |||
- [[Renal cell cancer]] | |||
- [[Pheochromocytoma]] | |||
- Uterine [[Leiomyoma|leiomyomas]] | |||
- Exogenous EPO: | |||
- Drug associated | |||
- EPO 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><ref name="pmid20008248">{{cite journal| author=McMullin MF| title=Idiopathic erythrocytosis: a disappearing entity. | journal=Hematology Am Soc Hematol Educ Program | year= 2009 | volume= | issue= | pages= 629-35 | pmid=20008248 | doi=10.1182/asheducation-2009.1.629 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20008248 }}</ref><ref name="pmid11317965">{{cite journal| author=Pearson TC, Messinezy M| title=Idiopathic erythrocytosis, diagnosis and clinical management. | journal=Pathol Biol (Paris) | year= 2001 | volume= 49 | issue= 2 | pages= 170-7 | pmid=11317965 | doi=10.1016/s0369-8114(00)00025-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11317965 }}</ref> | |||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
[[Category: | [[Category:Hematology]] | ||
[[Category:Emergency medicine]] | |||
[[Category:Disease]] | |||
[[Category:Blood disorders]] |
Latest revision as of 17:26, 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][2][3][4]
- Primary erythrocytosis[5][6]
- Secondary erythrocytosis[7]
- Erythropoietin receptor-mediated - High oxygen affinity hemoglobin - Bisphosphoglycerate mutase deficiency - VHL (Von Hippel-Lindau) gene mutation (Chuvash erythrocytosis) - PHD2 (prolyl hydroxylase domain) 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 [8][9][10]
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.
- ↑ Pearson TC (1998). "Diagnosis and classification of erythrocytoses and thrombocytoses". Baillieres Clin Haematol. 11 (4): 695–720. doi:10.1016/s0950-3536(98)80035-8. PMID 10640213.
- ↑ Golde DW, Hocking WG, Koeffler HP, Adamson JW (1981). "Polycythemia: mechanisms and management". Ann Intern Med. 95 (1): 71–87. doi:10.7326/0003-4819-95-1-71. PMID 7018337.
- ↑ Erslev, A. J.; Caro, J. (2009). "Pathophysiology and Classification of Polycythaemia". Scandinavian Journal of Haematology. 31 (4): 287–292. doi:10.1111/j.1600-0609.1983.tb00655.x. ISSN 0036-553X.
- ↑ Messinezy M, Pearson TC (1999). "The classification and diagnostic criteria of the erythrocytoses (polycythaemias)". Clin Lab Haematol. 21 (5): 309–16. doi:10.1046/j.1365-2257.1999.00246.x. PMID 10646073.
- ↑ McMullin MF (2008). "The classification and diagnosis of erythrocytosis". Int J Lab Hematol. 30 (6): 447–59. doi:10.1111/j.1751-553X.2008.01102.x. PMID 18823397.
- ↑ Prchal JT (2003). "Classification and molecular biology of polycythemias (erythrocytoses) and thrombocytosis". Hematol Oncol Clin North Am. 17 (5): 1151–8, vi. doi:10.1016/s0889-8588(03)00090-x. PMID 14560779.
- ↑ 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.
- ↑ McMullin MF (2009). "Idiopathic erythrocytosis: a disappearing entity". Hematology Am Soc Hematol Educ Program: 629–35. doi:10.1182/asheducation-2009.1.629. PMID 20008248.
- ↑ Pearson TC, Messinezy M (2001). "Idiopathic erythrocytosis, diagnosis and clinical management". Pathol Biol (Paris). 49 (2): 170–7. doi:10.1016/s0369-8114(00)00025-0. PMID 11317965.