Preferred Label : Beta thalassaemia major;
ICD-11 definition : Beta-thalassemia major is a severe early-onset form of beta-thalassemia (see this
term) characterized by reduced (beta ) or absent (beta0) synthesis of the beta-chains
of hemoglobin (Hb) leading to severe anemia requiring regular red blood cell transfusions.;
ICD-11 synonym : Cooley anaemia;
ICD-11 inclusion : Homozygous beta thalassaemia; Compound heterozygotes for beta 0 or beta or delta-beta-thal genes;
Origin ID : 1583088290;
Currated CISMeF NLP mapping
- Validated automatic mappings to NTBT
Beta-thalassemia major is a severe early-onset form of beta-thalassemia (see this
term) characterized by reduced (beta ) or absent (beta0) synthesis of the beta-chains
of hemoglobin (Hb) leading to severe anemia requiring regular red blood cell transfusions.
