3A70.0

ICD-11 MMS 2026-01

Congenital aplastic anaemia

Description

A disease caused by determinants in the antenatal period leading to the inability of stem cells to generate new mature cells. This disease is characterised by low levels of red blood cells, white blood cells, platelets. This disease may present with pallor, fatigue, dizziness, increased risk of infection or increased bruising or bleeding.

Index terms

  • Congenital aplastic anaemia
  • Constitutional medullar aplasia
  • congenital or familial hypoplastic anaemia
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  • congenital pancytopenia
  • constitutional aplastic anaemia
  • constitutional aplastic anaemia with malformation
  • constitutional panhaematopenia
  • constitutional panhemocytopenia
  • familial hypoplastic anaemia
  • pancytopenia with malformation
  • Fanconi anaemia
  • Fanconi pancytopenia
  • Fanconi pancytopenia syndrome
  • Fanconi-ichthyosis-dysmorphism syndrome
  • Fanconi familial refractory anaemia
  • Fanconi hypoplastic anaemia
  • Dyskeratosis congenita
  • Autosomal dominant dyskeratosis congenita
  • AD-DKC - [Autosomal dominant dyskeratosis congenita]
  • Autosomal recessive dyskeratosis congenita
  • AR-DKC - [Autosomal recessive dyskeratosis congenita]
  • Hoyeraal-Hreidarsson syndrome
  • Progressive pancytopenia - immunodeficiency - cerebellar hypoplasia
  • Shwachman-Diamond syndrome
  • Congenital lipomatosis of pancreas
  • WT limb-blood syndrome

Inclusions

  • Constitutional medullar aplasia
  • familial hypoplastic anaemia
  • Fanconi anaemia

Excludes

  • 3B64.01 – Congenital amegakaryocytic thrombocytopenia

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

Foundation children elsewhere

  • KA8C – Congenital hypoplastic anaemia
  • LD2F.15 – Noonan syndrome

Sibling codes

  • 3A70.1 – Acquired aplastic anaemias
  • 3A70.Z – Aplastic anaemia, unspecified

Foundation entity

Congenital aplastic anaemia – http://id.who.int/icd/entity/350719523

Part of: 3A70: Aplastic anaemia › 03: Diseases of the blood or blood-forming organs