3A50.1

ICD-11 MMS 2026-01

Alpha thalassaemia related syndromes

Description

Alpha-thalassemia-related diseases refers to a group of diseases characterised by alpha-thalassemia and an associated disorder. Three conditions are included in this group: alpha-thalassemia-intellectual deficit syndrome, X-linked (or ATR-X syndrome), alpha-thalassemia-intellectual deficit syndrome and alpha-thalassemia-myelodysplastic disease or ATMDS.

Index terms

  • Alpha thalassaemia related syndromes
  • Alpha thalassaemia related diseases
  • Alpha thalassaemia - X-linked intellectual deficit
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  • X-linked alpha thalassaemia with mental retardation
  • Alpha thalassaemia - intellectual deficit syndrome
  • 16pter-p13.3 deletion
  • Alpha-thalassaemia intellectual disability syndrome linked to chromosome 16

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
  • 3A50.2 – Beta thalassaemia
  • 3A50.3 – Delta, delta-beta or gamma-delta-beta thalassaemia
  • 3A50.4 – Hereditary persistence of fetal haemoglobin
  • 3A50.Y – Other specified thalassaemias
  • 3A50.Z – Thalassaemias, unspecified

Foundation entity

Alpha thalassaemia related syndromes – http://id.who.int/icd/entity/675675654

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