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.
- 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