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Congenital Dyserythropoietic Anemia (Dyserythropoietic Anemia, Congenital)

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
Networked: 158 relevant articles (4 outcomes, 13 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Congenital Dyserythropoietic Anemia (Dyserythropoietic Anemia, Congenital)
2. Hyperplasia
3. Anemia
4. Iron Overload
5. beta-Thalassemia (Cooley's Anemia)

Experts

1. Iolascon, Achille: 14 articles (01/2022 - 10/2003)
2. Russo, Roberta: 11 articles (01/2022 - 05/2010)
3. Tamary, Hannah: 10 articles (01/2021 - 08/2002)
4. Andolfo, Immacolata: 6 articles (01/2022 - 01/2019)
5. Barcellini, Wilma: 6 articles (10/2021 - 09/2009)
6. Marra, Roberta: 5 articles (01/2022 - 01/2019)
7. Rosato, Barbara Eleni: 5 articles (01/2022 - 01/2019)
8. Shalev, Hanna: 5 articles (08/2020 - 10/2008)
9. Bieker, James J: 4 articles (12/2020 - 08/2013)
10. De Rosa, Gianluca: 4 articles (11/2020 - 01/2019)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Congenital Dyserythropoietic Anemia:
1. IronIBA
2. Interferon-alpha (Interferon Alfa)IBA
3. InterferonsIBA
4. Transferrin (beta 2 Transferrin)IBA
5. Proteins (Proteins, Gene)FDA Link
6. Glycoproteins (Glycoprotein)IBA
7. EnzymesIBA
8. Polysaccharides (Glycans)IBA
9. Membrane Proteins (Integral Membrane Proteins)IBA
10. ChromatinIBA
01/01/2000 - "Anisopoikilocytosis with tear-drop forms, polychromasia, basophilic stippling in peripheral blood smear, erythroid hyperplasia with megaloblastoid changes, binucleated cells and intranuclear bridging in bone marrow aspirate and spongy, unevenly condensed nuclear chromatin in electron microscopy studies indicated that he had congenital dyserythropoietic anemia (CDA) type I. As a rare finding in CDA, ringed sideroblasts were noted. "
03/23/2020 - "Congenital dyserythropoietic anemia type I (CDA I), is an autosomal recessive disease with macrocytic anemia in which erythroid precursors in the bone marrow exhibit pathognomonic abnormalities including spongy heterochromatin and chromatin bridges. "
06/23/2011 - "Congenital dyserythropoietic anemia type 1 (CDA-1), a rare inborn anemia characterized by abnormal chromatin ultrastructure in erythroblasts, is caused by abnormalities in codanin-1, a highly conserved protein of unknown function. "
10/01/2008 - "Congenital dyserythropoietic anemia type I (CDA I) is a rare inherited disease characterized by moderate to severe macrocytic anemia and abnormal erythroid precursors with nuclear chromatin bridges and spongy heterochromatin. "
06/01/2005 - "Congenital dyserythropoietic anemia type I (CDA I) is a rare inherited hematological disorder characterized by macrocytic anemia and ineffective erythropoiesis with pathognomonic morphological features that include internuclear chromatin bridges, spongy heterochromatin, and invagination of the cytoplasm into the nuclear area in erythroid precursors. "

Therapies and Procedures

1. Intravenous Infusions
2. Duration of Therapy
3. Lasers (Laser)
4. Splenectomy
5. Therapeutics