Preferred Label : Severe combined immunodeficiency with reticular dysgenesis;
ICD-11 definition : Reticular dysgenesis is the most severe form of severe combined immunodeficiency (SCID)
and is characterized by bilateral sensorineural deafness and a lack of innate and
adaptive immune functions leading to fatal septicemia within days after birth if not
treated.;
ICD-11 synonym : generalised haematopoietic hypoplasia; Congenital aleukocytosis; Reticular dysgenesis; Severe combined immunodeficiency with leukopenia; SCID with reticular dysgenesis; De Vaal disease; SCID - [Severe combined immunodeficiencies] with reticular dysgenesis;
Origin ID : 194355955;
Currated CISMeF NLP mapping
Reticular dysgenesis is the most severe form of severe combined immunodeficiency (SCID)
and is characterized by bilateral sensorineural deafness and a lack of innate and
adaptive immune functions leading to fatal septicemia within days after birth if not
treated.
