Congenital Haemolytic Anaemia – Definitions & ICD 11 Codes

Definition of Congenital Haemolytic Anaemia: A disease caused by determinants arising in the antenatal period . This disease is characterised by low levels of red blood cells in the body due to abnormal destruction of the red blood cells. This disease may present with pallor, fatigue, or shortness of breath. Confirmation is by identification of low red blood cell count in a blood sample.

Coded Elsewhere:

  • Haemolytic disease of fetus or newborn (KA84)

Hereditary haemolytic anaemia

ICD 11 Code For Hereditary haemolytic anaemia

  3A10  Hereditary haemolytic anaemia

3A10.0 Haemolytic anaemias due to hexose monophosphate shunt or glutathione metabolism anomalies

Definition of Haemolytic anaemias due to hexose monophosphate shunt or glutathione metabolism anomalies: This is a form of anaemia due to haemolysis, the abnormal breakdown of red blood cells (RBCs), either in the blood vessels (intravascular haemolysis) or elsewhere in the human body (extravascular). This diagnosis is due to is a process that generates NADPH and pentoses (5-carbon sugars) and glutathione metabolism anomalies.

3A10.00 Haemolytic anaemia due to glucose-6-phosphate dehydrogenase deficiency

Definition of Haemolytic anaemia due to glucose-6-phosphate dehydrogenase deficiency: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common hereditary erythrocyte enzyme deficiency that can manifest with severe neonatal jaundice which can lead to serious neurological consequences, or, most often, with acute haemolytic anaemia following ingestion of certain foods (fava beans), common drugs (some antimalaria drugs, sulphamides, analgesics), or in the course of an infection, in otherwise asymptomatic individuals.

3A10.0Y Other specified haemolytic anaemias due to hexose monophosphate shunt or glutathione metabolism anomalies

3A10.0Z Haemolytic anaemias due to hexose monophosphate shunt or glutathione metabolism anomalies unspecified

3A10.1 Haemolytic anaemia due to adenosine deaminase excess

3A10.2 Hereditary elliptocytosis

Definition of Hereditary elliptocytosis: Hereditary elliptocytosis is a group of rare conditions caused by abnormalities in the red cell cytoskeleton and marked by the presence on blood smears of numerous elliptical red blood cells, called elliptocytes. Clinical presentations are highly heterogeneous ranging from asymptomatic forms to more severe forms associated with variable anaemia, from moderate to severe and with pyropoikilocytosis including fragmented red cells, microelliptocytes and microspherocytes.

3A10.3 Familial pseudohyperkalaemia

Definition of Familial pseudohyperkalaemia: A disease caused by a genetically inherited mutation. This disease is characterised by a temperature-dependent defect in red cell membrane permeability to potassium that leads to high in vitro potassium levels in samples stored below 37°C leading to elevated potassium levels in the blood that does not reflect the true potassium level. Confirmation is by identification of genetic mutation through genetic testing.

3A10.Y Other specified hereditary haemolytic anaemia

3A10.Z Hereditary haemolytic anaemia unspecified

Other specified congenital haemolytic anaemia

ICD 11 Code For Other specified congenital haemolytic anaemia

  3A1Y  Other specified congenital haemolytic anaemia

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