ICD-11 code : 3A21.2;
Preferred Label : Haemolytic uraemic syndrome;
ICD-11 definition : A disease caused by acquired haematopoietic stem cell mutation defect of the PIGA
gene in the X chromosome leading to the premature break down of red blood cells. This
disease is characterised by haemolytic anaemia, thrombosis and. This disease may present
with haemoglobinuria (blood in the urine will be most noticeable in the morning, and
clear as the day progresses), pallor, fatigue, or shortness of breath. Confirmation
is by identification of genetic mutation in the PIGA gene through genetic testing.;
ICD-11 synonym : Gasser syndrome; Typical haemolytic uraemic syndrome;
Origin ID : 630790515;
UMLS CUI : C0019061;
Automatic exact mappings (from CISMeF team)
Currated CISMeF NLP mapping
ICD-10 Mapping
Semantic type(s)
UMLS correspondences (same concept)
A disease caused by acquired haematopoietic stem cell mutation defect of the PIGA
gene in the X chromosome leading to the premature break down of red blood cells. This
disease is characterised by haemolytic anaemia, thrombosis and. This disease may present
with haemoglobinuria (blood in the urine will be most noticeable in the morning, and
clear as the day progresses), pallor, fatigue, or shortness of breath. Confirmation
is by identification of genetic mutation in the PIGA gene through genetic testing.