Childhood red cell aplasia is defined as anemia, with reticulocytopenia, and usually bone marrow erythroid hypoplasia. The congenital disorder is called Diamond-Blackfan anemia. The acquired disease is short-lived, termed transient erythroblastopenia of childhood. The onset of Diamond-Blackfan anemia is usually prior to age 2 years. One-third have some physical abnormality. Erythroid progenitor cells are decreased in number and relatively insensitive to erythropoietin in tissue culture. Steroid treatment leads to clinical response in two-thirds. During steroid-induced or spontaneous remission, patients demonstrate fetal erythropoiesis. TEC occurs between ages 1 and 6 years. Physical examinations are normal. Spontaneous remissions occur rapidly. Erythrocytes are initially adult in character; fetal erythrocytes appear transiently during the recovery phase. Full recovery is expected.
|Original language||English (US)|
|Number of pages||19|
|Journal||American Journal of Pediatric Hematology/Oncology|
|State||Published - 1980|
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health