Congenital dyserythropoietic anaemia type II (CDA-II): chromosomal banding studies and adherent cell effects on erythroid colony (CFU-E) and burst (BFU-E) formation. 1982

G D Roodman, and C N Clare, and G Mills

Bone marrow CFU-E and BFU-E from a patient with CDA-II formed erythroid colonies and bursts which contained multinucleated erythroblasts in vitro. Adherent cell depletion of the patient's marrow increased CFU-E derived colonies six-fold (98 +/- 17 v. 640 +/- 15 per 10(5) marrow cells plated) and co-culture of CDA-II marrow adherent cells with CSA-II adherent cell depleted marrow significantly suppressed erythroid colony formation. Similar adherent cell suppression of the patient's BFU-E also occurred. Adherent cell depletion of normal marrow did not increase CFU-E derived colony formation (488 +/- 63 v. 495 +/- 108) and decreased BFU-E derived burst formation. Addition of normal adherent cells to normal marrow increased erythroid colony and burst formation. Karyotype and chromosomal banding studies of the patient's multinucleated cells did not show chromosomal inversions, deletions or translocations.

UI MeSH Term Description Entries
D008297 Male Males
D001853 Bone Marrow The soft tissue filling the cavities of bones. Bone marrow exists in two types, yellow and red. Yellow marrow is found in the large cavities of large bones and consists mostly of fat cells and a few primitive blood cells. Red marrow is a hematopoietic tissue and is the site of production of erythrocytes and granular leukocytes. Bone marrow is made up of a framework of connective tissue containing branching fibers with the frame being filled with marrow cells. Marrow,Red Marrow,Yellow Marrow,Marrow, Bone,Marrow, Red,Marrow, Yellow
D002448 Cell Adhesion Adherence of cells to surfaces or to other cells. Adhesion, Cell,Adhesions, Cell,Cell Adhesions
D002478 Cells, Cultured Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others. Cultured Cells,Cell, Cultured,Cultured Cell
D002871 Chromosome Banding Staining of bands, or chromosome segments, allowing the precise identification of individual chromosomes or parts of chromosomes. Applications include the determination of chromosome rearrangements in malformation syndromes and cancer, the chemistry of chromosome segments, chromosome changes during evolution, and, in conjunction with cell hybridization studies, chromosome mapping. Banding, Chromosome,Bandings, Chromosome,Chromosome Bandings
D004920 Erythropoiesis The production of red blood cells (ERYTHROCYTES). In humans, erythrocytes are produced by the YOLK SAC in the first trimester; by the liver in the second trimester; by the BONE MARROW in the third trimester and after birth. In normal individuals, the erythrocyte count in the peripheral blood remains relatively constant implying a balance between the rate of erythrocyte production and rate of destruction. Erythropoieses
D006412 Hematopoietic Stem Cells Progenitor cells from which all blood cells derived. They are found primarily in the bone marrow and also in small numbers in the peripheral blood. Colony-Forming Units, Hematopoietic,Progenitor Cells, Hematopoietic,Stem Cells, Hematopoietic,Hematopoietic Progenitor Cells,Cell, Hematopoietic Progenitor,Cell, Hematopoietic Stem,Cells, Hematopoietic Progenitor,Cells, Hematopoietic Stem,Colony Forming Units, Hematopoietic,Colony-Forming Unit, Hematopoietic,Hematopoietic Colony-Forming Unit,Hematopoietic Colony-Forming Units,Hematopoietic Progenitor Cell,Hematopoietic Stem Cell,Progenitor Cell, Hematopoietic,Stem Cell, Hematopoietic,Unit, Hematopoietic Colony-Forming,Units, Hematopoietic Colony-Forming
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

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