" /> Beta thalassaemia - CISMeF





ICD-11 code : 3A50.2;

Preferred Label : Beta thalassaemia;

ICD-11 definition : Beta-thalassemia (BT) is a haemoglobinopathy characterised by deficiency (Beta ) or absence (Beta0) of synthesis of the beta globin chains of haemoglobin (Hb). Three main types of BT have been described: minor, intermedia and major with clinical presentation ranging from asymptomatic forms to microcytic anaemia and splenomegaly due to defective erythropiesis and haemolysis.;

ICD-11 synonym : Mediterranean anaemia; Mediterranean disease; beta thalassaemia syndrome;

ICD-11 inclusion : erythroblastic anaemia of childhood; familial erythroblastic anaemia; beta thalassaemia disease; severe beta thalassaemia;

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Beta-thalassemia (BT) is a haemoglobinopathy characterised by deficiency (Beta ) or absence (Beta0) of synthesis of the beta globin chains of haemoglobin (Hb). Three main types of BT have been described: minor, intermedia and major with clinical presentation ranging from asymptomatic forms to microcytic anaemia and splenomegaly due to defective erythropiesis and haemolysis.

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18/05/2024


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