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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
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</ref> 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 pplycythemia</nowiki> according the hematology guidelines.</ref> 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] [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
Shown below is an algorithm summarizing the treatment of [[disease name]] according the the [...] guidelines.
Do's
- The content in this section is in bullet points.
Don'ts
- The content in this section is in bullet points.
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.