3A50.4

ICD-11 MMS 2026-01

Hereditary persistence of fetal haemoglobin

Description

Hereditary persistence of fetal haemoglobin (HPFH) associated with beta-thalassaemia is a haemoglobinopathy characterised by high haemoglobin (Hb)F levels and an increased number of fetal-Hb-containing cells. The association of HPFH with beta-thalassaemia mitigates the clinical manifestations which vary from a normal state to beta-thalassaemia intermedia.

Index terms

  • Hereditary persistence of fetal haemoglobin
  • HPFH - [Hereditary persistence of fetal haemoglobin]
  • fetal haemoglobin
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  • persistence of fetal haemoglobin
  • persistent haemoglobin F
  • Hereditary persistence of fetal haemoglobin, deletional
  • Hereditary persistence of fetal haemoglobin, non-deletional

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

  • 3A50.0 – Alpha thalassaemia
  • 3A50.1 – Alpha thalassaemia related syndromes
  • 3A50.2 – Beta thalassaemia
  • 3A50.3 – Delta, delta-beta or gamma-delta-beta thalassaemia
  • 3A50.Y – Other specified thalassaemias
  • 3A50.Z – Thalassaemias, unspecified

Foundation entity

Hereditary persistence of fetal haemoglobin – http://id.who.int/icd/entity/418601307

Part of: 3A50: Thalassaemias › 03: Diseases of the blood or blood-forming organs