3A51.A

ICD-11 MMS 2026-01

Haemoglobin E disease

Description

Haemoglobin E disease is characterised by the synthesis of an abnormal haemoglobin called haemoglobin E (HbE), instead of the normal haemoglobin A (HbA). Subjects heterozygous for HbE (AE) have an asymptomatic condition with no clinical relevance, except for the risk of transmitting E/beta thalassemia if the other parent carries beta thalassemia. The severity of these E/beta thalassemia forms is very variable, the clinical picture ranging from that of beta thalassemia minor through to thalassemia intermedia to thalassemia major. Subjects homozygous for HbE (EE) are asymptomatic.

Index terms

  • Haemoglobin E disease
  • Homozygous HbE carriers
  • Compound HbE or other Hb mutant heterozygotes

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

  • 3A51.0 – Sickle cell trait
  • 3A51.1 – Sickle cell disease without crisis
  • 3A51.2 – Sickle cell disease with crisis
  • 3A51.3 – Compound heterozygous sickling disorders without crisis
  • 3A51.4 – Compound heterozygous sickling disorders with crisis
  • 3A51.5 – Haemoglobin C disease
  • 3A51.6 – Haemoglobin D disease
  • 3A51.7 – High affinity haemoglobin
  • 3A51.8 – Low affinity haemoglobin
  • 3A51.9 – Haemoglobin O disease
  • 3A51.B – Haemoglobin C/beta thalassaemia compound heterozygosity
  • 3A51.Y – Other specified sickle cell disorders or other haemoglobinopathies
  • 3A51.Z – Sickle cell disorders or other haemoglobinopathies, unspecified

Foundation entity

Haemoglobin E disease – http://id.who.int/icd/entity/1898135714

Part of: 3A51: Sickle cell disorders or other haemoglobinopathies › 03: Diseases of the blood or blood-forming organs