Polycythemia resident survival guide

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Fatigue
Resident Survival Guide
Overview
Causes
FIRE
Diagnosis
Treatment
Do's
Don'ts

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

Overview

Polycythemia is defined as increasing the hemoglobin (>16.5 g/dl in men and >16 g/dl in women) or hematocrit level (>49%in men and >48% in women).[1] 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). [2]

Causes

Life Threatening Causes

Common Causes

Primary polycythemia
Secondary polycythemia
  • Chronic lung disease
  • High altitude
  • EPO-producing tumors
  • High carboxyhemoglobin: mostly observed in smokers
  • Kidney diseases, such as Renal cysts and renal artery stenosis,
  • Iatrogenic reasons: steroids, erythropoietin treatment, anabolic testosterone replacement therapy. 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). [2]

Diagnosis

Shown below is an algorithm summarizing the diagnosis of polycythemia according to hematology guidelines. [2] [3] [4]

 
 
 
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
 
 
 

Treatment

Shown below is an algorithm summarizing the treatment of polycythemia vera. [5]


 
 
 
 
 
 
 
 
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

Do's

  • First, you should differentiate between absolute polycythemia and relative polycythemia
  • Ask the patient about using diuretics and products athletes use to improve their function
  • Low-dose of Aspirin must be prescribed for all cases of polycythemia vera who do not have any contraindication [6] [3]

Don'ts

  • Do not analyze JAK2 mutation in every case as a first step when there is no clinical finding related to polycythemia vera
  • In women with polycythemia vera do not consider pregnancy as a contraindication
  • Aspirin should not be prescribed in cases of acquired von Willebrand disease [7] [8]

References

  1. Barbui T, Thiele J, Gisslinger H, Kvasnicka HM, Vannucchi AM, Guglielmelli P; et al. (2018). "The 2016 WHO classification and diagnostic criteria for myeloproliferative neoplasms: document summary and in-depth discussion". Blood Cancer J. 8 (2): 15. doi:10.1038/s41408-018-0054-y. PMC 5807384. PMID 29426921.
  2. 2.0 2.1 2.2 Pillai AA, Fazal S, Babiker HM. PMID 30252337. Missing or empty |title= (help)
  3. 3.0 3.1 3.2 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.
  4. Jameson, J (2018). Harrison's principles of internal medicine. New York: McGraw-Hill Education. ISBN 978-1259643996.
  5. 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.
  6. 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.
  7. 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.
  8. 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.


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