3A73
Congenital dyserythropoietic anaemia
Anemia diseritropoética congênita
CategoryDefinition
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.
Exclusions
- Blackfan-Diamond syndrome
- Di Guglielmo disease
Index Terms
Congenital dyserythropoietic anaemiacongenital dyshaematopoietic anaemiaCDA - [congenital dyserythropoietic anaemia]dyserythropoietic dyshaematopoietic congenital anaemiadyshaematopoietic anaemiadyserythropoietic anaemiaCongenital dyserythropoietic anaemia type ICDA type 1 -[Congenital dyserythropoietic anaemia type 1]Congenital dyserythropoietic anaemia type IICDA type 2 - [Congenital dyserythropoietic anaemia type 2]Congenital dyserythropoietic anaemia type IIICDA type 3 - [Congenital dyserythropoietic anaemia type 3]Dyserythropoietic anaemia with thrombocytopeniaX-linked dyserythropoietic anaemia and thrombocytopenia