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Non-spherocytic hemolytic anemia due to hexokinase deficiency

ORPHA:90031· ICD-10 D55.2

Definition

Nonspherocytic haemolytic anaemia due to hexokinase deficiency is characterised by severe hemolysis, appearing in infancy. Seventeen affected families have been reported so far. Transmission is autosomal recessive. Mutations have been described in HK1, the gene that encodes red blood cell-specific hexokinase-R.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal recessive
Age of onset
Infancy