Polycythemia resident survival guide
| Fatigue Resident Survival Guide |
|---|
| Overview |
| Causes |
| 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, Approach to high red blood cell count
Overview
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).
Causes
Life Threatening Causes
- Critical dehydration owing to fluid loss such as severe diarrhea or vomiting, which can result in Spurious Polycythemia
- Severe cyanotic heart diseases with right-to-left shunts
- End-stage cancer related to EPO-secreting tumors such as hepatocellular carcinoma, parathyroid carcinoma, pheochromocytoma, or renal cell carcinoma [1]
Common Causes
- Primary polycythemia [2]
- Polycythemia vera and its complications
- 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 Causes:
- Steroids
- Erythropoietin treatment
- Anabolic testosterone replacement therapy
Diagnosis
Shown below is an algorithm summarizing the diagnosis of polycythemia according to hematology guidelines. [2] [1] [3]
| 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
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 | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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 [9] [1]
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 [10] [11]
References
- ↑ 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.
- ↑ 2.0 2.1 Pillai AA, Fazal S, Babiker HM. PMID 30252337. Missing or empty
|title=(help) - ↑ Jameson, J (2018). Harrison's principles of internal medicine. New York: McGraw-Hill Education. ISBN 978-1259643996.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.