Polycythemia resident survival guide
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Alieh Behjat, M.D.[2]
Overview
Polycythemia is defined as increasing the hemoglobin (>16.5 g/dl in men or >16 g/dl in women) or hematocrit level (>49%in men or >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
- 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[3]
Common Causes
- Primary polycythemia
- 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 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 according to new guidelines.
Do's
Don'ts
References
- ↑ 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.
- ↑ 2.0 2.1 2.2 Pillai AA, Fazal S, Babiker HM. PMID 30252337. Missing or empty
|title=(help) - ↑ 3.0 3.1 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.
- ↑ Jameson, J (2018). Harrison's principles of internal medicine. New York: McGraw-Hill Education. ISBN 978-1259643996.