Haemolytic anaemias due to hexose monophosphate shunt or glutathione metabolism anomalies

Description

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 a process that generates NADPH and pentoses (5-carbon sugars) and glutathione metabolism anomalies.

Exclusions from above levels

04 – Diseases of the immune system
05 – Endocrine, nutritional or metabolic diseases
18 – Complications of pregnancy, childbirth or the puerperium

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19 – Certain conditions originating in the perinatal period
20 – Congenital malformations, deformations or chromosomal abnormalities
22 – Injury, poisoning or certain other consequences of external causes
http://id.who.int/icd/entity/1000704511 – Human immunodeficiency virus disease
JB64.1 – Other diseases of the blood or blood-forming organs or certain disorders involving the immune mechanism complicating pregnancy, childbirth or the puerperium

Child codes

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

Sibling codes

3A10.1 – Haemolytic anaemia due to adenosine deaminase excess
3A10.2 – Hereditary elliptocytosis
3A10.3 – Familial pseudohyperkalaemia
3A10.Y – Other specified hereditary haemolytic anaemia
3A10.Z – Hereditary haemolytic anaemia, unspecified

Foundation entity

Haemolytic anaemias due to hexose monophosphate shunt or glutathione metabolism anomalies – http://id.who.int/icd/entity/2071787420

Part of: 3A10: Hereditary haemolytic anaemia › 03: Diseases of the blood or blood-forming organs