Primary inherited erythrocytosis

Description

A disease caused by genetically inherited factors leading to changes in the concentration of red blood cells. This disease is characterised by having a high concentration of red blood cells in the body leading to slow flow of blood. Confirmation is by identification of mutations by genetic testing.

Index terms

Primary inherited erythrocytosis
Primary familial polycythaemia
Primary inherited polycythaemia

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Polycythaemia due to HIF2A mutation
Polycythaemia due to PHD2 mutation
Polycythaemia due to VHL mutation
Chuvash erythrocytosis
Chuvash polycythaemia
Von Hippel-Lindau-dependent polycythaemia
Primary familial polycythaemia due to EPOR mutation
Primary familial polycythaemia due to other mutations

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

Sibling codes

3A80.Y – Other specified congenital polycythaemia
3A80.Z – Congenital polycythaemia, unspecified

Foundation entity

Primary inherited erythrocytosis – http://id.who.int/icd/entity/962836252

Part of: 3A80: Congenital polycythaemia › 03: Diseases of the blood or blood-forming organs