Polycythemia vera risk factors: Difference between revisions
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{{Polycythemia vera}} | {{Polycythemia vera}} | ||
{{CMG}} {{AE}}{{MJK}} | {{CMG}} {{AE}} {{IO}} {{MJK}} {{shyam}} | ||
==Overview== | ==Overview== | ||
Common risk factors in the development of polycythemia vera are old age (65 year old and older), family history, and Ashkenazi Jewish descent. After a patient is diagnosed with polycythemia vera, risk stratification involves assessment of age and thrombotic history. The risk assessment for development of post-PV myelofibrosis or post-PV acute myeloid leukemia include a variety of factors. | Common risk factors in the development of polycythemia vera are old age (65 year old and older), family history, and Ashkenazi Jewish descent. After a patient is diagnosed with polycythemia vera, risk stratification involves assessment of age and thrombotic history. The risk assessment for development of post-PV [[myelofibrosis]] or post-PV [[acute myeloid leukemia]] include a variety of factors. | ||
==Risk Factors== | ==Risk Factors== | ||
Risk factors in the development of polycythemia vera are:<ref name="pmid25377561">{{cite journal| author=Barbui T, Carobbio A, Rumi E, Finazzi G, Gisslinger H, Rodeghiero F et al.| title=In contemporary patients with polycythemia vera, rates of thrombosis and risk factors delineate a new clinical epidemiology. | journal=Blood | year= 2014 | volume= 124 | issue= 19 | pages= 3021-3 | pmid=25377561 | doi=10.1182/blood-2014-07-591610 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25377561 }} </ref> | Risk factors in the development of polycythemia vera are:<ref name="pmid25377561">{{cite journal| author=Barbui T, Carobbio A, Rumi E, Finazzi G, Gisslinger H, Rodeghiero F et al.| title=In contemporary patients with polycythemia vera, rates of thrombosis and risk factors delineate a new clinical epidemiology. | journal=Blood | year= 2014 | volume= 124 | issue= 19 | pages= 3021-3 | pmid=25377561 | doi=10.1182/blood-2014-07-591610 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25377561 }} </ref> | ||
*'''Old age (65 years older)''' | *'''Old age (65 years older)''' | ||
*'''Family history of polycythemia vera''' | **[[mutations]] can accumulate in [[hematopoietic stem cells]] over time, resulting in a higher probability of a [[Janus kinase|JAK2]] mutation. | ||
*'''Ashkenazi Jewish descent''' | *'''Family history of polycythemia vera''' | ||
**First-degree relatives of patients with polycythemia vera have a 5 to 7-fold higher risk for development of a [[myeloproliferative neoplasm]]. | |||
**The cumulative risk of developing [[myelofibrosis]] is 6% at 10 years, 14% at 15 years, and 26% at 20 years from the initial diagnosis of polycythemia vera.<ref name="pmid27884974">{{cite journal| author=Vannucchi AM| title=From leeches to personalized medicine: evolving concepts in the management of polycythemia vera. | journal=Haematologica | year= 2017 | volume= 102 | issue= 1 | pages= 18-29 | pmid=27884974 | doi=10.3324/haematol.2015.129155 | pmc=5210229 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27884974 }} </ref> | |||
*'''Ashkenazi Jewish descent''' | |||
==Risk Stratification== | ==Risk Stratification== | ||
| Line 21: | Line 24: | ||
*History of prior [[thrombosis]] | *History of prior [[thrombosis]] | ||
*[[Leukocytosis]] | *[[Leukocytosis]] | ||
*Increased [[Janus kinase|JAK2]] V617F allele burden | *Increased [[Janus kinase|JAK2]] V617F [[Alleles|allele]] burden | ||
*High risk gene expression profile | *High risk [[gene expression]] profile | ||
The risk factors for the transformation to myelofibrosis or secondary [[acute myeloid leukemia]] include:<ref name="pmid26324368">{{cite journal |vauthors=Stein BL, Oh ST, Berenzon D, Hobbs GS, Kremyanskaya M, Rampal RK, Abboud CN, Adler K, Heaney ML, Jabbour EJ, Komrokji RS, Moliterno AR, Ritchie EK, Rice L, Mascarenhas J, Hoffman R |title=Polycythemia Vera: An Appraisal of the Biology and Management 10 Years After the Discovery of JAK2 V617F |journal=J. Clin. Oncol. |volume=33 |issue=33 |pages=3953–60 |date=November 2015 |pmid=26324368 |pmc=4979103 |doi=10.1200/JCO.2015.61.6474 |url=}}</ref> | The risk factors for the transformation to [[myelofibrosis]] or secondary [[acute myeloid leukemia]] include:<ref name="pmid26324368">{{cite journal |vauthors=Stein BL, Oh ST, Berenzon D, Hobbs GS, Kremyanskaya M, Rampal RK, Abboud CN, Adler K, Heaney ML, Jabbour EJ, Komrokji RS, Moliterno AR, Ritchie EK, Rice L, Mascarenhas J, Hoffman R |title=Polycythemia Vera: An Appraisal of the Biology and Management 10 Years After the Discovery of JAK2 V617F |journal=J. Clin. Oncol. |volume=33 |issue=33 |pages=3953–60 |date=November 2015 |pmid=26324368 |pmc=4979103 |doi=10.1200/JCO.2015.61.6474 |url=}}</ref> | ||
*Older age | *Older age | ||
*Extended disease duration | *Extended disease duration | ||
| Line 30: | Line 33: | ||
*Exposure to [[phosphorus-32]], [[pipobroman]], or [[chlorambucil]] | *Exposure to [[phosphorus-32]], [[pipobroman]], or [[chlorambucil]] | ||
The risk factors associated with decreased survival include:<ref name="pmid26324368">{{cite journal |vauthors=Stein BL, Oh ST, Berenzon D, Hobbs GS, Kremyanskaya M, Rampal RK, Abboud CN, Adler K, Heaney ML, Jabbour EJ, Komrokji RS, Moliterno AR, Ritchie EK, Rice L, Mascarenhas J, Hoffman R |title=Polycythemia Vera: An Appraisal of the Biology and Management 10 Years After the Discovery of JAK2 V617F |journal=J. Clin. Oncol. |volume=33 |issue=33 |pages=3953–60 |date=November 2015 |pmid=26324368 |pmc=4979103 |doi=10.1200/JCO.2015.61.6474 |url=}}</ref> | The risk factors associated with decreased [[Survival rates|survival]] include:<ref name="pmid26324368">{{cite journal |vauthors=Stein BL, Oh ST, Berenzon D, Hobbs GS, Kremyanskaya M, Rampal RK, Abboud CN, Adler K, Heaney ML, Jabbour EJ, Komrokji RS, Moliterno AR, Ritchie EK, Rice L, Mascarenhas J, Hoffman R |title=Polycythemia Vera: An Appraisal of the Biology and Management 10 Years After the Discovery of JAK2 V617F |journal=J. Clin. Oncol. |volume=33 |issue=33 |pages=3953–60 |date=November 2015 |pmid=26324368 |pmc=4979103 |doi=10.1200/JCO.2015.61.6474 |url=}}</ref> | ||
*Older age | *Older age | ||
*[[Leukocytosis]] | *[[Leukocytosis]] | ||
*History of [[venous thrombosis]] | *History of [[venous thrombosis]] | ||
*Abnormal karyotype | *Abnormal [[karyotype]] | ||
==References== | ==References== | ||
Latest revision as of 03:03, 14 September 2019
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ifeoma Odukwe, M.D. [2] Mohamad Alkateb, MBBCh [3] Shyam Patel [4]
Overview
Common risk factors in the development of polycythemia vera are old age (65 year old and older), family history, and Ashkenazi Jewish descent. After a patient is diagnosed with polycythemia vera, risk stratification involves assessment of age and thrombotic history. The risk assessment for development of post-PV myelofibrosis or post-PV acute myeloid leukemia include a variety of factors.
Risk Factors
Risk factors in the development of polycythemia vera are:[1]
- Old age (65 years older)
- mutations can accumulate in hematopoietic stem cells over time, resulting in a higher probability of a JAK2 mutation.
- Family history of polycythemia vera
- First-degree relatives of patients with polycythemia vera have a 5 to 7-fold higher risk for development of a myeloproliferative neoplasm.
- The cumulative risk of developing myelofibrosis is 6% at 10 years, 14% at 15 years, and 26% at 20 years from the initial diagnosis of polycythemia vera.[2]
- Ashkenazi Jewish descent
Risk Stratification
High risk features of polycythemia vera include:
- Old age
- History of thrombotic event
- Presence of JAK2 mutation
The risk factors for the development of thrombosis include:[3]
- Age >60 years
- History of prior thrombosis
- Leukocytosis
- Increased JAK2 V617F allele burden
- High risk gene expression profile
The risk factors for the transformation to myelofibrosis or secondary acute myeloid leukemia include:[3]
- Older age
- Extended disease duration
- Leukocytosis
- Exposure to phosphorus-32, pipobroman, or chlorambucil
The risk factors associated with decreased survival include:[3]
- Older age
- Leukocytosis
- History of venous thrombosis
- Abnormal karyotype
References
- ↑ Barbui T, Carobbio A, Rumi E, Finazzi G, Gisslinger H, Rodeghiero F; et al. (2014). "In contemporary patients with polycythemia vera, rates of thrombosis and risk factors delineate a new clinical epidemiology". Blood. 124 (19): 3021–3. doi:10.1182/blood-2014-07-591610. PMID 25377561.
- ↑ Vannucchi AM (2017). "From leeches to personalized medicine: evolving concepts in the management of polycythemia vera". Haematologica. 102 (1): 18–29. doi:10.3324/haematol.2015.129155. PMC 5210229. PMID 27884974.
- ↑ 3.0 3.1 3.2 Stein BL, Oh ST, Berenzon D, Hobbs GS, Kremyanskaya M, Rampal RK, Abboud CN, Adler K, Heaney ML, Jabbour EJ, Komrokji RS, Moliterno AR, Ritchie EK, Rice L, Mascarenhas J, Hoffman R (November 2015). "Polycythemia Vera: An Appraisal of the Biology and Management 10 Years After the Discovery of JAK2 V617F". J. Clin. Oncol. 33 (33): 3953–60. doi:10.1200/JCO.2015.61.6474. PMC 4979103. PMID 26324368.