Polycythemia resident survival guide

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Resident Survival Guide

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Alieh Behjat, M.D.[2]

Synonyms and keywords: Approach to polycythemia, Approach to high hemoglobin level, Approach to high red blood cell count


Polycythemia is defined as high hemoglobin (>16.5 g/dl in men and >16 g/dl in women) or hematocrit level (>49%in men and >48% in women). This elevated level might be due to declining the plasma volume (relative or spurious polycythemia) or rising the number of red blood cells (true polycythemia).


Life Threatening Causes

Common Causes

Primary polycythemia [2]
Secondary polycythemia


Shown below is an algorithm summarizing the diagnosis of polycythemia according to hematology guidelines. [2] [1] [3]

Abbreviations: Hgb: Hemoglobin, Hct: Hematocrit RBC: Red blood cell, EPO: Erythropoietin, COPD: Chronic obstructive pulmonary disease

Elevated Hgb or Hct
Assess RBC mass
if normal
Relative erythrocytosis
If high level
Measure EPO level
If low
Polycythemia vera
Check JAK2 mutation to confirm
If high level
Assess arterial O2 saturation
If low
Assess cardiac or pulmunary diseases, such as right to left shunts, COPD, high altitute
If normal
Is the paitient smoker?
If no
Measure Hgb O2 affinity
If normal
Diagnostic evaluation for finding tumor producing EPO: Kidney sonography, Brain CT, Abdominopelvic Ct scan
If incresed
High oxigen affinity hemoglobinopathy
If yes
Evaluate carboxihemoglobin levels
If normal
If high
Smoker's polycythemia


Curing the underlying causes of secondary polycythemia is the primary way of treatment in this disease. Shown below is an algorithm summarizing the treatment of polycythemia vera. [4] [5] [6] [7] [8]

The main treatment in Polycythemia Vera
Phlebotomy in order to keep hematocrit lower than 45% and prescribe Aspirin(40-100 mg) once every day
Low-risk patient (without any history of thrombosis and ≤60 years)
High-risk patient (with a history of thrombosis or older than 60 years )
If a patient has microvascular symptoms, which have not been controlled sufficiently or leukocytosis or cardiovascular symptoms specifically hypertension
Add hydroxyurea with the initial dose of 500 mg twice daily
If the patient could not tolerate hydroxyurea or was resistant to it
Prescribe Aspirin BID
If the patient has a history of arterial thrombosis
If the patient has a history of venous trombosis
prescribe Pegylated IFN-α or Busulfan
Prescribe Aspirin BID
Add systemic anticoagulant therapy




  1. 1.0 1.1 1.2 McMullin, Mary F.; Bareford, D.; Campbell, P.; Green, A. R.; Harrison, Claire; Hunt, Beverley; Oscier, D.; Polkey, M. I.; Reilly, J. T.; Rosenthal, E.; Ryan, Kate; Pearson, T. C.; Wilkins, Bridget (2005). "Guidelines for the diagnosis, investigation and management of polycythaemia/erythrocytosis". British Journal of Haematology. 130 (2): 174–195. doi:10.1111/j.1365-2141.2005.05535.x. ISSN 0007-1048.
  2. 2.0 2.1 Pillai AA, Fazal S, Babiker HM. PMID 30252337. Missing or empty |title= (help)
  3. Jameson, J (2018). Harrison's principles of internal medicine. New York: McGraw-Hill Education. ISBN 978-1259643996.
  4. Tefferi, Ayalew; Vannucchi, Alessandro M.; Barbui, Tiziano (2018). "Polycythemia vera treatment algorithm 2018". Blood Cancer Journal. 8 (1). doi:10.1038/s41408-017-0042-7. ISSN 2044-5385.
  5. Marchioli, Roberto; Finazzi, Guido; Specchia, Giorgina; Cacciola, Rossella; Cavazzina, Riccardo; Cilloni, Daniela; De Stefano, Valerio; Elli, Elena; Iurlo, Alessandra; Latagliata, Roberto; Lunghi, Francesca; Lunghi, Monia; Marfisi, Rosa Maria; Musto, Pellegrino; Masciulli, Arianna; Musolino, Caterina; Cascavilla, Nicola; Quarta, Giovanni; Randi, Maria Luigia; Rapezzi, Davide; Ruggeri, Marco; Rumi, Elisa; Scortechini, Anna Rita; Santini, Simone; Scarano, Marco; Siragusa, Sergio; Spadea, Antonio; Tieghi, Alessia; Angelucci, Emanuele; Visani, Giuseppe; Vannucchi, Alessandro Maria; Barbui, Tiziano (2013). "Cardiovascular Events and Intensity of Treatment in Polycythemia Vera". New England Journal of Medicine. 368 (1): 22–33. doi:10.1056/NEJMoa1208500. ISSN 0028-4793.
  6. Quintás-Cardama, Alfonso; Abdel-Wahab, Omar; Manshouri, Taghi; Kilpivaara, Outi; Cortes, Jorge; Roupie, Anne-Laure; Zhang, Su-Jiang; Harris, David; Estrov, Zeev; Kantarjian, Hagop; Levine, Ross L.; Verstovsek, Srdan (2013). "Molecular analysis of patients with polycythemia vera or essential thrombocythemia receiving pegylated interferon α-2a". Blood. 122 (6): 893–901. doi:10.1182/blood-2012-07-442012. ISSN 0006-4971.
  7. Barbui, Tiziano; Masciulli, Arianna; Marfisi, Maria Rosa; Tognoni, Giovanni; Finazzi, Guido; Rambaldi, Alessandro; Vannucchi, Alessandro (2015). "White blood cell counts and thrombosis in polycythemia vera: a subanalysis of the CYTO-PV study". Blood. 126 (4): 560–561. doi:10.1182/blood-2015-04-638593. ISSN 0006-4971.
  8. Barbui, Tiziano; Vannucchi, Alessandro M.; Carobbio, Alessandra; Rumi, Elisa; Finazzi, Guido; Gisslinger, Heinz; Ruggeri, Marco; Randi, Maria Luigia; Cazzola, Mario; Rambaldi, Alessandro; Gisslinger, Bettina; Pieri, Lisa; Thiele, Juergen; Pardanani, Animesh; Tefferi, Ayalew (2017). "The effect of arterial hypertension on thrombosis in low-risk polycythemia vera". American Journal of Hematology. 92 (1): E5–E6. doi:10.1002/ajh.24583. ISSN 0361-8609.
  9. van Genderen PJ, Mulder PG, Waleboer M, van de Moesdijk D, Michiels JJ (April 1997). "Prevention and treatment of thrombotic complications in essential thrombocythaemia: efficacy and safety of aspirin". Br. J. Haematol. 97 (1): 179–84. doi:10.1046/j.1365-2141.1997.d01-2127.x. PMID 9136963.
  10. Maze, Dawn; Kazi, Sajida; Gupta, Vikas; Malinowski, Ann Kinga; Fazelzad, Rouhi; Shah, Prakesh S.; Shehata, Nadine (2019). "Association of Treatments for Myeloproliferative Neoplasms During Pregnancy With Birth Rates and Maternal Outcomes". JAMA Network Open. 2 (10): e1912666. doi:10.1001/jamanetworkopen.2019.12666. ISSN 2574-3805.
  11. Tefferi, Ayalew; Barbui, Tiziano (2017). "Polycythemia vera and essential thrombocythemia: 2017 update on diagnosis, risk-stratification, and management". American Journal of Hematology. 92 (1): 94–108. doi:10.1002/ajh.24607. ISSN 0361-8609.