Polycythemia vera diagnostic criteria: Difference between revisions
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==Overview== | ==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|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. | 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== | ||
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*Endogenous erythroid colony growth | *Endogenous erythroid colony growth | ||
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*Diagnosis requires the presence of both major criteria plus 1 minor criteron, OR the first major criterion and 2 minor criteria | *Diagnosis requires the presence of both major criteria plus 1 minor criteron, OR the first major criterion and 2 minor criteria. | ||
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*Diagnosis requires the presence of all 3 major criteria, or the first 2 major criteria plus 1 minor criteria | *Diagnosis requires the presence of all 3 major criteria, or the first 2 major criteria plus 1 minor criteria | ||
<sub> | <sub>[[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.</sub> | ||
==References== | ==References== | ||
Revision as of 22:26, 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] |
|---|---|
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Major criteria |
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Minor criteria |
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- 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 |
|---|---|
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Major criteria |
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Minor criteria |
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- 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
- ↑ 1.0 1.1 Tefferi A, Thiele J, Orazi A, Kvasnicka HM, Barbui T, Hanson CA; et al. (2007). "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". Blood. 110 (4): 1092–7. doi:10.1182/blood-2007-04-083501. PMID 17488875.
- ↑ Tefferi A, Thiele J, Vardiman JW (2009). "The 2008 World Health Organization classification system for myeloproliferative neoplasms: order out of chaos". Cancer. 115 (17): 3842–7. doi:10.1002/cncr.24440. PMID 19472396.
- ↑ Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM; et al. (2016). "The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia". Blood. 127 (20): 2391–405. doi:10.1182/blood-2016-03-643544. PMID 27069254.