" /> Haemolytic uraemic syndrome - CISMeF





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;

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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.

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01/05/2025


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