| Polycythaemia vera | |
|---|---|
| Other names | Polycythaemia vera, erythremia, primary polycythemia, Vaquez disease, Osler-Vaquez disease, polycythemia rubra vera[1] |
 | |
| Blood smear from a person with polycythemia vera | |
| Specialty | Oncology |
| Symptoms | Itchiness, headaches, blurry vision, burning pain in the hands or feet, reddish skin[2] |
| Complications | Thrombosis, myelofibrosis, hyperviscosity syndrome, leukemia[2] |
| Usual onset | 60 years[2] |
| Duration | Long-term[3] |
| Diagnostic method | CBC, bone marrow biopsy, genetic testing after ruling out other causes of polycythemia[2] |
| Differential diagnosis | Other causes of polycythemia, essential thrombocythemia, chronic myelogenous leukemia[2] |
| Treatment | Removing blood, aspirin[2] |
| Prognosis | Life expectancy ~14 years with treatment[2] |
| Frequency | 5 per 10,000[3] |
Polycythemia vera (PCV) is a disorder in which the bone marrow makes too many red blood cells, and occasionally white blood cells and platelets.[2] Symptoms may include itchiness, headaches, blurry vision, burning pain in the hands or feet, and reddish skin.[2] Complications can include blood clots, myelofibrosis, hyperviscosity syndrome, and leukemia.[2]
More than 90% of cases are due to a mutation of the Janus kinase 2 (JAK2) gene in a group of blood forming cells.[2] This occurs after birth and for an unclear reason.[3] This results in increased sensitivity to growth factors.[2] It is a type of myeloproliferative neoplasm.[2] Diagnosis is based based on a complete blood cell count, bone marrow biopsy, and genetic testing after ruling out other causes of polycythemia.[2]
Treatment consists removing blood and aspirin.[2] Other measures include a healthy lifestyle.[2] In older people or those with a higher risk of blood clots hydroxyurea, interferon, busulfan may be recommended.[2] Occationally removal of the spleen is required.[2] Life expectancy is around 18 months without treatment and 14 years with treatment.[2]
The condition is uncommon, affected about 5 per 10,000.[3] The typical age of onset is 60 and the condition is rare under the age of 20.[2][3] Males and females are affected with similar frequency.[2] The condition was first described in 1892.[3]
References edit
- ^ "polycythemia vera." at Encyclopædia Britannica. 2010. Encyclopædia Britannica Online. 21 Sep. 2010
- ^ a b c d e f g h i j k l m n o p q r s t u Lu, X; Chang, R (January 2020). "Polycythemia Vera". PMID 32491592.
{{cite journal}}: Cite journal requires|journal=(help) - ^ a b c d e f "Polycythemia Vera". NORD (National Organization for Rare Disorders). Retrieved 7 January 2021.