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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
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 the 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 [[disease name]] according the the [...] guidelines.

<nowiki>
 
 
 
 
 
 
 
 
The main treatment in polycythemia Vera
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Phlebotomy in order to keep hematocrit lower than 45%
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Low-risk patient(•without any history of thrombosis and •≤60 years)
 
 
 
 
 
 
 
 
 
 
 
High-risk patient
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
D01'
D01
 
 
 
 
 
 
 
 
 
 
 
D02'
D02
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
E01
 
 
 
 
 
 
 
E02
 
E03
 
 
E04
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
F01
 
F02
 
 
F03

Do's

  • The content in this section is in bullet points.

Don'ts

  • The content in this section is in bullet points.

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 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.


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