ICD-11 classes
03 Diseases of the blood or blood-forming organs
Anaemias or other erythrocyte disorders
3A73 — Congenital dyserythropoietic anaemia
ICD-11 3A73 — Congenital dyserythropoietic anaemia
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.
The diagnosis includes nothing.
It excludes 2 items.
- Blackfan-Diamond syndrome (3A60.1)
- Di Guglielmo disease (2A60.35)
It has no clarifying diagnoses.