Article

[Childhood essential polycythemia: an unusual disorder].

Services de médecine infantile et d'hématologie biologique, CHU de Nancy.
Annales de biologie clinique (impact factor: 0.34). 63(1):83-7. pp.83-7
Source: PubMed

ABSTRACT We report the case of an 2-year-old boy presenting an essential polycythemia since birth, with details of the diagnostic procedures used and clinical course. Pediatric cases are very rare, and a secondary acquired polycythemia should be first investigated. Most causes of primary childhood polycythemia remains unknown. Erythropoietin (EPO) level may help to separate diseases with high EPO (Chuvash, or yet unclassified), or with normal/low EPO (congenital with truncation of the EPO receptor, polycythemia vera-Vaquez disease-, or currently with unknown mechanism).

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Keywords

2-year-old boy
 
causes
 
Chuvash
 
congenital
 
diagnostic procedures
 
EPO
 
EPO receptor
 
normal/low EPO
 
Pediatric cases
 
polycythemia vera-Vaquez disease-
 
secondary
 
separate diseases
 
unknown
 
unknown mechanism