3A73

ICD-11 MMS 2026-01

Congenital dyserythropoietic anaemia

Description

Congenital dyserythropoietic anaemias (CDA) result from diverse erythropoietic disorders; they lead to the defective production of red blood cells (RBC) and often mild haemolysis that attests to a qualitative defect of these RBC released into the circulation. Three forms of CDA have been characterised: types I, II and III. The shared symptoms include anaemia of variable severity, intermittent jaundice, splenomegaly and hepatomegaly.

Index terms

  • Congenital dyserythropoietic anaemia
  • CDA - [congenital dyserythropoietic anaemia]
  • congenital dyshaematopoietic anaemia
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  • dyserythropoietic anaemia
  • dyserythropoietic dyshaematopoietic congenital anaemia
  • dyshaematopoietic anaemia
  • Congenital dyserythropoietic anaemia type I
  • CDA type 1 -[Congenital dyserythropoietic anaemia type 1]
  • Congenital dyserythropoietic anaemia type II
  • CDA type 2 - [Congenital dyserythropoietic anaemia type 2]
  • Congenital dyserythropoietic anaemia type III
  • CDA type 3 - [Congenital dyserythropoietic anaemia type 3]
  • Dyserythropoietic anaemia with thrombocytopenia
  • X-linked dyserythropoietic anaemia and thrombocytopenia

Excludes

  • 2A60.35 – Di Guglielmo disease
  • 3A60.1 – Blackfan-Diamond syndrome

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 – Thalassaemias
  • 3A51 – Sickle cell disorders or other haemoglobinopathies
  • 3A70 – Aplastic anaemia
  • 3A71 – Anaemia due to chronic disease
  • 3A72 – Sideroblastic anaemia
  • 3A90 – Anaemia due to acute disease
  • 3A91 – Congenital methaemoglobinaemia
  • 3A92 – Hereditary methaemoglobinaemia
  • 3A93 – Acquired methaemoglobinaemia
  • 3A94 – Acute posthaemorrhagic anaemia
  • 3A9Y – Other specified anaemias or erythrocyte disorders
  • 3A9Z – Anaemias or other erythrocyte disorders, unspecified

Foundation entity

Congenital dyserythropoietic anaemia – http://id.who.int/icd/entity/899830967

Part of: 03: Diseases of the blood or blood-forming organs