Biomaterial Database

[Congenital dyserythropoietic anemia type III. A case report]. 1997

T Holm, and O Kildahl-Andersen, and M Sjo

Sykestua, Asegarden militaerleir.

Congenital dyserythropoietic anaemia type III is a rare disorder characterized by mild to moderate anaemia, ineffective erythropoiesis, and morphologic abnormalities of mature red blood cells and their precursors. The most extraordinary feature of this condition is the large number of multinuclear erythroblasts found in the bone marrow, some containing up to 12 nuclei. This type of anaemia is an autosomal dominant inherited disorder, though sporadic cases have been described. Little conclusive is known about the pathogenesis of congenital dyserthropoietic anaemia type III. At present the management of patients consists of observation and supportive care. We describe a 20 year old man who was admitted to a county hospital, showing the typical features of this rare illness. He had a Hb value of 10.2 g/100 ml.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D000742	Anemia, Dyserythropoietic, 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.	Dyserythropoietic Anemia, Congenital,HEMPAS,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,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