Polycythemia classification: Difference between revisions

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==Overview==
==Overview==
There is no established system for the classification of [disease name].
Mainly classified into primary and secondary causes based on the presence or absence of [[genetic mutations]] and underlying disorders.
 
OR
 
[Disease name] may be classified according to [classification method] into [number] subtypes/groups: [group1], [group2], [group3], and [group4].
 
OR
 
[Disease name] may be classified into [large number > 6] subtypes based on [classification method 1], [classification method 2], and [classification method 3].
[Disease name] may be classified into several subtypes based on [classification method 1], [classification method 2], and [classification method 3].
 
OR
 
Based on the duration of symptoms, [disease name] may be classified as either acute or chronic.
 
OR
 
If the staging system involves specific and characteristic findings and features:
According to the [staging system + reference], there are [number] stages of [malignancy name] based on the [finding1], [finding2], and [finding3]. Each stage is assigned a [letter/number1] and a [letter/number2] that designate the [feature1] and [feature2].
 
OR
 
The staging of [malignancy name] is based on the [staging system].
 
OR
 
There is no established system for the staging of [malignancy name].


==Classification==
==Classification==


There is no established system for the classification of [disease name].
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>
 
OR
 
[Disease name] may be classified according to [classification method] into [number] subtypes/groups:
*[Group1]
*[Group2]
*[Group3]
*[Group4]
 
OR
 
[Disease name] may be classified into [large number > 6] subtypes based on:
*[Classification method 1]
*[Classification method 2]
*[Classification method 3]
 
[Disease name] may be classified into several subtypes based on:  
*[Classification method 1]
*[Classification method 2]
*[Classification method 3]
 
OR
 
Based on the duration of symptoms, [disease name] may be classified as either acute or chronic.
 
OR
 
'''If the staging system involves specific and characteristic findings and features:'''
 
According to the [staging system + reference], there are [number] stages of [malignancy name] based on the [finding1], [finding2], and [finding3]. Each stage is assigned a [letter/number1] and a [letter/number2] that designate the [feature1] and [feature2].


OR
*'''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>


The staging of [malignancy name] is based on the [staging system].
- [[Polycythemia vera]]


OR
*'''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>
 
There is no established system for the staging of [malignancy name].


[[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:Hematology|Hematology]]
[[Category:Hematology]]
 
[[Category:Emergency medicine]]
{{WS}}
[[Category:Disease]]
{{WH}}
[[Category:Blood disorders]]

Latest revision as of 17:26, 25 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:[1][2][3][4]

- Polycythemia vera

Congenital: 

         - 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

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 [8][9][10]

References

  1. ↑ 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.
  2. ↑ 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.
  3. ↑ 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.
  4. ↑ 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.
  5. ↑ 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.
  6. ↑ 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.
  7. ↑ 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.
  8. ↑ 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.
  9. ↑ 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.
  10. ↑ 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.
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