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Polycythemia (Excluding Polycythemia Vera)

MedGen UID:
163107
•Concept ID:
C0852343
•
Disease or Syndrome
Synonym: Polycythemia (Excluding Rubra Vera)

Definition

An increase in the concentration of red blood cells (hemoglobin) that is not caused by polycythemia vera. [from NCI]

Professional guidelines

PubMed

Diagnosis and management of neutrophilic myeloid neoplasms.
Schwede M, Gotlib J, Shomali W
Clin Adv Hematol Oncol 2021 Jul;19(7):450-459. PMID: 34236344
Polycythemia vera and essential thrombocythemia: 2021 update on diagnosis, risk-stratification and management.
Tefferi A, Barbui T
Am J Hematol 2020 Dec;95(12):1599-1613. Epub 2020 Oct 23 doi: 10.1002/ajh.26008. PMID: 32974939
Polycythemia vera and essential thrombocythemia: 2019 update on diagnosis, risk-stratification and management.
Tefferi A, Barbui T
Am J Hematol 2019 Jan;94(1):133-143. Epub 2018 Nov 9 doi: 10.1002/ajh.25303. PMID: 30281843

Recent clinical studies

Etiology

Lab tests for MPN.
Moncada A, Pancrazzi A
Int Rev Cell Mol Biol 2022;366:187-220. Epub 2021 Apr 5 doi: 10.1016/bs.ircmb.2021.02.010. PMID: 35153004
Polycythemia vera and essential thrombocythemia: 2021 update on diagnosis, risk-stratification and management.
Tefferi A, Barbui T
Am J Hematol 2020 Dec;95(12):1599-1613. Epub 2020 Oct 23 doi: 10.1002/ajh.26008. PMID: 32974939
Polycythemia vera and essential thrombocythemia: 2019 update on diagnosis, risk-stratification and management.
Tefferi A, Barbui T
Am J Hematol 2019 Jan;94(1):133-143. Epub 2018 Nov 9 doi: 10.1002/ajh.25303. PMID: 30281843
Management of symptoms in polycythemia vera and essential thrombocythemia patients.
Radia D, Geyer HL
Hematology Am Soc Hematol Educ Program 2015;2015:340-8. doi: 10.1182/asheducation-2015.1.340. PMID: 26637741
Polycythemia vera.
Stuart BJ, Viera AJ
Am Fam Physician 2004 May 1;69(9):2139-44. PMID: 15152961

Diagnosis

Diagnosis and management of neutrophilic myeloid neoplasms.
Schwede M, Gotlib J, Shomali W
Clin Adv Hematol Oncol 2021 Jul;19(7):450-459. PMID: 34236344
Polycythemia vera and essential thrombocythemia: 2021 update on diagnosis, risk-stratification and management.
Tefferi A, Barbui T
Am J Hematol 2020 Dec;95(12):1599-1613. Epub 2020 Oct 23 doi: 10.1002/ajh.26008. PMID: 32974939
Polycythemia vera and essential thrombocythemia: 2019 update on diagnosis, risk-stratification and management.
Tefferi A, Barbui T
Am J Hematol 2019 Jan;94(1):133-143. Epub 2018 Nov 9 doi: 10.1002/ajh.25303. PMID: 30281843
Polycythemia vera.
Stuart BJ, Viera AJ
Am Fam Physician 2004 May 1;69(9):2139-44. PMID: 15152961
Polycythemia vera.
Murphy S
Dis Mon 1992 Mar;38(3):153-212. doi: 10.1016/0011-5029(92)90002-7. PMID: 1587207

Therapy

Polycythemia vera and essential thrombocythemia: 2021 update on diagnosis, risk-stratification and management.
Tefferi A, Barbui T
Am J Hematol 2020 Dec;95(12):1599-1613. Epub 2020 Oct 23 doi: 10.1002/ajh.26008. PMID: 32974939
Properties of FDA-approved small molecule protein kinase inhibitors: A 2020 update.
Roskoski R Jr
Pharmacol Res 2020 Feb;152:104609. Epub 2019 Dec 17 doi: 10.1016/j.phrs.2019.104609. PMID: 31862477
Polycythemia vera and essential thrombocythemia: 2019 update on diagnosis, risk-stratification and management.
Tefferi A, Barbui T
Am J Hematol 2019 Jan;94(1):133-143. Epub 2018 Nov 9 doi: 10.1002/ajh.25303. PMID: 30281843
Management of symptoms in polycythemia vera and essential thrombocythemia patients.
Radia D, Geyer HL
Hematology Am Soc Hematol Educ Program 2015;2015:340-8. doi: 10.1182/asheducation-2015.1.340. PMID: 26637741
Polycythemia vera and essential thrombocythemia: 2012 update on diagnosis, risk stratification, and management.
Tefferi A
Am J Hematol 2012 Mar;87(3):285-93. doi: 10.1002/ajh.23135. PMID: 22331582

Prognosis

High platelet-to-lymphocyte ratio may differentiate polycythemia vera from secondary polycythemia.
Krečak I, Holik H, Morić Perić M, Zekanović I, Coha B, Gverić-Krečak V, Lucijanić M
Wien Klin Wochenschr 2022 Jun;134(11-12):483-486. Epub 2022 Apr 7 doi: 10.1007/s00508-022-02027-w. PMID: 35391561
Lab tests for MPN.
Moncada A, Pancrazzi A
Int Rev Cell Mol Biol 2022;366:187-220. Epub 2021 Apr 5 doi: 10.1016/bs.ircmb.2021.02.010. PMID: 35153004
Diagnosis and management of neutrophilic myeloid neoplasms.
Schwede M, Gotlib J, Shomali W
Clin Adv Hematol Oncol 2021 Jul;19(7):450-459. PMID: 34236344
Polycythemia vera and essential thrombocythemia: 2021 update on diagnosis, risk-stratification and management.
Tefferi A, Barbui T
Am J Hematol 2020 Dec;95(12):1599-1613. Epub 2020 Oct 23 doi: 10.1002/ajh.26008. PMID: 32974939
Management of symptoms in polycythemia vera and essential thrombocythemia patients.
Radia D, Geyer HL
Hematology Am Soc Hematol Educ Program 2015;2015:340-8. doi: 10.1182/asheducation-2015.1.340. PMID: 26637741

Clinical prediction guides

Lab tests for MPN.
Moncada A, Pancrazzi A
Int Rev Cell Mol Biol 2022;366:187-220. Epub 2021 Apr 5 doi: 10.1016/bs.ircmb.2021.02.010. PMID: 35153004
Impact of comorbidities and body mass index on the outcome of polycythemia vera patients.
Benevolo G, Elli EM, Bartoletti D, Latagliata R, Tiribelli M, Heidel FH, Cavazzini F, Bonifacio M, Crugnola M, Binotto G, D'Addio A, Tieghi A, Bergamaschi M, Caocci G, Polverelli N, Bossi E, Auteri G, Carmosino I, Catani L, Cuneo A, Krampera M, Lanza F, Lemoli RM, Vianelli N, Breccia M, Palumbo GA, Cavo M, Palandri F
Hematol Oncol 2021 Aug;39(3):409-418. Epub 2021 Mar 8 doi: 10.1002/hon.2843. PMID: 33590502
The 2001 World Health Organization and updated European clinical and pathological criteria for the diagnosis, classification, and staging of the Philadelphia chromosome-negative chronic myeloproliferative disorders.
Michiels JJ, De Raeve H, Berneman Z, Van Bockstaele D, Hebeda K, Lam K, Schroyens W
Semin Thromb Hemost 2006 Jun;32(4 Pt 2):307-40. doi: 10.1055/s-2006-942754. PMID: 16810609
Chronic myeloproliferative disorders.
Spivak JL, Barosi G, Tognoni G, Barbui T, Finazzi G, Marchioli R, Marchetti M
Hematology Am Soc Hematol Educ Program 2003:200-24. doi: 10.1182/asheducation-2003.1.200. PMID: 14633783
Aquagenic pruritus.
Steinman HK, Greaves MW
J Am Acad Dermatol 1985 Jul;13(1):91-6. doi: 10.1016/s0190-9622(85)70149-1. PMID: 2411768

Recent systematic reviews

The utility of testing erythropoietin level in polycythemia diagnosis.
Ismail A, Abdalla E, Aqel A, Fadul A, Ahmed A, Alsayed A, Musa M, Yassin MA
Hematology 2023 Dec;28(1):2269510. Epub 2023 Oct 16 doi: 10.1080/16078454.2023.2269510. PMID: 37843428
Pharmacokinetics and Pharmacodynamics of Ruxolitinib: A Review.
Appeldoorn TYJ, Munnink THO, Morsink LM, Hooge MNL, Touw DJ
Clin Pharmacokinet 2023 Apr;62(4):559-571. Epub 2023 Mar 31 doi: 10.1007/s40262-023-01225-7. PMID: 37000342Free PMC Article
Coexistent antiphospholipid syndrome and myeloproliferative neoplasm.
Sayar Z, Nallamilli S, Efthymiou M, Lambert J, Cohen H
Lupus 2021 Aug;30(9):1502-1508. Epub 2021 Jul 1 doi: 10.1177/09612033211021154. PMID: 34192956

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