Congenital Dyserythropoietic Anemia

A familial disorder characterized by ANEMIA with multinuclear ERYTHROBLASTS, karyorrhexis, asynchrony of nuclear and cytoplasmic maturation, and various nuclear abnormalities of bone marrow erythrocyte precursors (ERYTHROID PRECURSOR CELLS). Type II is the most common of the 3 types; it is often referred to as HEMPAS, based on the Hereditary Erythroblast Multinuclearity with Positive Acidified Serum test.

Also Known As:

Dyserythropoietic Anemia, Congenital; Anemia, Dyserythropoietic, Congenital; Anemia With Multinucleated Erythroblasts; Anemia, Congenital Dyserythropoietic; Anemia, Congenital Dyserythropoietic, Type II; Anemia, Dyserythropoietic Congenital, Type I; Anemia, Dyserythropoietic Congenital, Type II; Anemia, Dyserythropoietic Congenital, Type III; Anemia, Dyserythropoietic, Congenital Type 1; Anemia, Dyserythropoietic, Congenital Type 2; Anemia, Dyserythropoietic, Congenital, Type I; Anemia, Dyserythropoietic, Congenital, Type II; Anemia, Dyserythropoietic, Congenital, Type III; Congenital Dyserythropoietic Anemia Type 1; Congenital Dyserythropoietic Anemia Type II; Congenital Dyserythropoietic Anemia, Type I; Congenital Dyserythropoietic Anemia, Type III; Dyserythropoietic Anemia, Congenital Type 1; Dyserythropoietic Anemia, Congenital Type 2; Dyserythropoietic Anemia, Congenital, Type I; Dyserythropoietic Anemia, Congenital, Type II; Dyserythropoietic Anemia, Congenital, Type III; Dyserythropoietic Anemia, HEMPAS Type; HEMPAS Anemia; Hereditary Erythroblast Multinuclearity with Positive Acidified Serum; Hereditary Erythroblastic Multinuclearity with Positive Acidified-Serum Test; Type I Congenital Dyserythropoietic Anemia; Anemias, Congenital Dyserythropoietic; Congenital Dyserythropoietic Anemias; Dyserythropoietic Anemias, Congenital; HEMPAS Anemias; HEMPAS

Hemic and Lymphatic Diseases: 6
- Hematologic Diseases: 3366
  - Anemia: 38331
    - Hemolytic Anemia: 6695
      - Congenital Hemolytic Anemia: 288
        Congenital Dyserythropoietic Anemia: 158
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 933
- Inborn Genetic Diseases: 11939
  - Congenital Hemolytic Anemia: 288
    - Congenital Dyserythropoietic Anemia: 158

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Drug/Important Bio-Agent (IBA)