Polycythemia vera diagnostic criteria: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Polycythemia vera}} | {{Polycythemia vera}} | ||
{{CMG}}{{AE}}{{MJK}} | {{CMG}} {{AE}} {{MJK}}; {{shyam}} | ||
==Overview== | ==Overview== | ||
The diagnosis of polycythemia vera is based on the | The diagnosis of polycythemia vera is based on the World Health Organization criteria, which was initially proposed in 2008 then revised in 2016. In general, the diagnosis of polycythemia vera requires a combination of elevated [[hemoglobin]], which include high levels of [[hemoglobin]], presence of ''[[JAK2|JAK2 V617F]]'' mutation, hypercellularity on [[bone marrow]] biopsy, low serum [[erythropoietin]] level, and [[endogenous]] [[erythroid]] colony formation ''in vitro''.<ref name="pmid17488875">{{cite journal| author=Tefferi A, Thiele J, Orazi A, Kvasnicka HM, Barbui T, Hanson CA et al.| title=Proposals and rationale for revision of the World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis: recommendations from an ad hoc international expert panel. | journal=Blood | year= 2007 | volume= 110 | issue= 4 | pages= 1092-7 | pmid=17488875 | doi=10.1182/blood-2007-04-083501 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17488875 }} </ref> THe 2016 WHO criteria more accurately reflect the disease biology of polycythemia vera. | ||
==Diagnostic Criteria== | ==Diagnostic Criteria== | ||
{| style="border: 0px; font-size: 90%; margin: 3px; width: 600px" align=center | In 2008, the World Health Organization first proposed formal criteria for the diagnosis of polycythemia vera. | ||
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! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Category}} | ! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Category}} | ||
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Diagnostic criteria}} | ! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|2008 WHO Diagnostic criteria<ref name="pmid19472396">{{cite journal| author=Tefferi A, Thiele J, Vardiman JW| title=The 2008 World Health Organization classification system for myeloproliferative neoplasms: order out of chaos. | journal=Cancer | year= 2009 | volume= 115 | issue= 17 | pages= 3842-7 | pmid=19472396 | doi=10.1002/cncr.24440 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19472396 }} </ref>}} | ||
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Major criteria | Major criteria | ||
| style="padding: 5px 5px; background: #F5F5F5;" | | | style="padding: 5px 5px; background: #F5F5F5;" | | ||
*Hemoglobin > 18.5 g/dL in men, 16.5 g/dL in women or | *Hemoglobin > 18.5 g/dL in men, 16.5 g/dL in women, OR Hemoglobin > 17 g/dL in men, 15 g/dL in women is associated with a sustained increase of greater than or equal to 2 grams per deciliter from the baseline that cannot be attributed to correction of iron deficiency | ||
*Presence of ''[[JAK2| | *Presence of ''[[JAK2|JAK2 V617F]]'', JAK2 exon 12 mutation, or other functionally similar mutation such as ''[[JAK2]]'' exon 12 mutation | ||
|- | |- | ||
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" | | | style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" | | ||
Minor criteria | Minor criteria | ||
| style="padding: 5px 5px; background: #F5F5F5;" | | | style="padding: 5px 5px; background: #F5F5F5;" | | ||
*Serum erythropoietin level below the reference range for normal | *Serum erythropoietin level below the reference range for normal | ||
*Endogenous erythroid colony | *Bone marrow biopsy showing hypercellularity for age with trilineage growth (panmyelosis) including prominent erythroid, granulocyte, and megakaryocyte proliferation with pleomorphic, mature megakaryocytes | ||
*Endogenous erythroid colony growth | |||
|} | |} | ||
*Diagnosis requires the presence of both major criteria | *Diagnosis requires the presence of both major criteria plus 1 minor criteron, OR the first major criterion and 2 minor criteria. | ||
The diagnosis of polycythemia vera in the current era is based on the 2016 World Health Organization criteria.<ref name="pmid27069254">{{cite journal| author=Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM et al.| title=The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. | journal=Blood | year= 2016 | volume= 127 | issue= 20 | pages= 2391-405 | pmid=27069254 | doi=10.1182/blood-2016-03-643544 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27069254 }} </ref> This criteria was revised from the criteria proposed in 2008, as the new criteria better reflect the biology of the disease. Importantly, more people will be diagnosed with polycythemia vera given that the threshold for [[hemoglobin]] elevation has been reduced for both men and women.<ref name="pmid17488875">{{cite journal| author=Tefferi A, Thiele J, Orazi A, Kvasnicka HM, Barbui T, Hanson CA et al.| title=Proposals and rationale for revision of the World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis: recommendations from an ad hoc international expert panel. | journal=Blood | year= 2007 | volume= 110 | issue= 4 | pages= 1092-7 | pmid=17488875 | doi=10.1182/blood-2007-04-083501 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17488875 }} </ref> | |||
{| style="border: 0px; font-size: 90%; margin: 3px; width: 600px" align="center" | |||
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! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Category}} | |||
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|2016 WHO Diagnostic criteria}} | |||
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Major criteria | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*Hemoglobin > 16.5 g/dL in men, 16.0 g/dL in women OR hematocrit 49% in men, 48% in women OR increased red cell mass* | |||
*Bone marrow biopsy showing hypercellularity for age with trilineage growth (panmyelosis) including prominent erythroid, granulocyte, and megakaryocyte proliferation with pleomorphic, mature megakaryocytes | |||
*Presence of ''[[JAK2|JAK2 V617F]]'', JAK2 exon 12 mutation, or other functionally similar mutation such as ''[[JAK2]]'' exon 12 mutation | |||
|- | |||
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" | | |||
Minor criteria | |||
| style="padding: 5px 5px; background: #F5F5F5;" | | |||
*Serum erythropoietin level below the reference range for normal. | |||
*Diagnosis requires the presence of all 3 major criteria, or the first 2 major criteria plus 1 minor criteria. | |||
*[[Hemoglobin]] or [[hematocrit]] greater than 99th percentile of method-specific reference range for age, sex, altitude of residence or [[hemoglobin]] greater than 17 g/dL in men, 15 g/dL in women if associated with a documented and sustained increase of at least 2 g/dL from an individual's baseline value that can not be attributed to correction of iron deficiency, or elevated red cell mass greater than 25% above mean normal predicted value. | |||
==References== | ==References== | ||
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{{WikiDoc Help Menu}} | {{WikiDoc Help Menu}} | ||
{{WikiDoc Sources}} | {{WikiDoc Sources}} | ||
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[[Category:Hematology]] | |||
Latest revision as of 22:28, 21 January 2019
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamad Alkateb, MBBCh [2]; Shyam Patel [3]
Overview
The diagnosis of polycythemia vera is based on the World Health Organization criteria, which was initially proposed in 2008 then revised in 2016. In general, the diagnosis of polycythemia vera requires a combination of elevated hemoglobin, which include high levels of hemoglobin, presence of JAK2 V617F mutation, hypercellularity on bone marrow biopsy, low serum erythropoietin level, and endogenous erythroid colony formation in vitro.[1] THe 2016 WHO criteria more accurately reflect the disease biology of polycythemia vera.
Diagnostic Criteria
In 2008, the World Health Organization first proposed formal criteria for the diagnosis of polycythemia vera.
| Category | 2008 WHO Diagnostic criteria[2] |
|---|---|
|
Major criteria |
|
|
Minor criteria |
|
- Diagnosis requires the presence of both major criteria plus 1 minor criteron, OR the first major criterion and 2 minor criteria.
The diagnosis of polycythemia vera in the current era is based on the 2016 World Health Organization criteria.[3] This criteria was revised from the criteria proposed in 2008, as the new criteria better reflect the biology of the disease. Importantly, more people will be diagnosed with polycythemia vera given that the threshold for hemoglobin elevation has been reduced for both men and women.[1]
| Category | 2016 WHO Diagnostic criteria |
|---|---|
|
Major criteria |
|
|
Minor criteria |
References
|