Polycythemia resident survival guide
| 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]
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). 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
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). [1]
Diagnosis
Shown below is an algorithm summarizing the diagnosis of polycythemia according to hematology guidelines. [1] [2]
| 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.
| 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
- Firs, 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 [2]
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
References
- ↑ 1.0 1.1 Pillai AA, Fazal S, Babiker HM. PMID 30252337. Missing or empty
|title=(help) - ↑ 2.0 2.1 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.