Condition

Congenital dyserythropoietic anemia

Diseases of the Blood and Immune Mechanism

Congenital dyserythropoietic anemia, or CDA, is an umbrella term for a group of rare inherited blood disorders in which the bone marrow only partly succeeds in producing healthy red blood cells, leading to anemia that can be severe. Symptoms depend on the type and severity and can include tiredness, weakness and jaundice, with an enlarged liver or spleen in some patients. The body may also absorb too much iron, which can damage other organs. Four types, CDA-1 to CDA-4, are generally recognised, each with its own genetic cause and bone marrow changes, and diagnosis needs a bone marrow sample examined under a microscope followed by genetic sequencing. Mild cases may need no treatment, while severe cases are managed with regular transfusions or stem cell transplantation. The name was coined in 1968, and fewer than a thousand cases had been described by 2024.

Facts
Classification
ICD-10 Chapter
III: Diseases of the Blood and Blood-Forming Organs and Certain Disorders Involving the Immune Mechanism 1
Communicable or Non-communicable
Non-Communicable 2
Causative Agent
Causative Agent (category)
Genetic / Hereditary 3
Prevalence Band
Rare (Under 1 in 2000) 4
Sources
1. Monarch Initiative: congenital dyserythropoietic anemia - Monarch Initiative MONDO:0019403
Monarch InitiativeCross reference: ICD10CM
Quote, Cross reference: ICD10CM
ICD10CM:D64.4
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2. MedlinePlus Genetics (National Library of Medicine): Congenital dyserythropoietic anemia - MedlinePlus Genetics
MedlinePlus Genetics (National Library of Medicine)Causes
Quote, Causes
Variants (also called mutations) in multiple genes have been found to cause CDA.
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3. Congenital dyserythropoietic anemia (Wikipedia)
Lead
Quote, Lead
All have genetic origin, usually inherited from parents
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4. Orphadata (Orphanet): Orphadata: Epidemiology of rare diseases (en_product9_prev)
Orphadata (Orphanet)ORPHA:85
Quote, ORPHA:85
Prevalence at birth Value and class 1-9 / 1 000 000
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