[Studies on the pathogenesis of congenital hypoplastic anemia (Diamond-Blackfan syndrome)]. 1986

K Sawada

CFU-E growth from fractionated bone marrow cells of five patients with Diamond Blackfan syndrome was studied. In all patients, CFU-E growth was reduced in mononuclear cell rich fraction. In two patients out of five, CFU-E growth was returned to normal by the depletion of E-rosette forming cells of monocytes from mononuclear cell rich fraction. In the patient whose CFU-E growth returned to normal by the depletion of E-rosette forming cells, cocultivation between bone marrow buffy coat cells and autologous bone marrow E-rosette forming cells resulted in a significant decrease of CFU-E growth, and there was a significant increase in CFU-E growth by the treatment of OKT 4. In other three patients out of five, there was no significant increase in CFU-E growth by the depletion of monocytes and/or E-rosette forming cells. It is concluded that immunologic causes such as cellular factors may play a role, at least in part, on the pathogenesis of Diamond-Blackfan syndrome.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D008297 Male Males
D009000 Monocytes Large, phagocytic mononuclear leukocytes produced in the vertebrate BONE MARROW and released into the BLOOD; contain a large, oval or somewhat indented nucleus surrounded by voluminous cytoplasm and numerous organelles. Monocyte
D001772 Blood Cell Count The number of LEUKOCYTES and ERYTHROCYTES per unit volume in a sample of venous BLOOD. A complete blood count (CBC) also includes measurement of the HEMOGLOBIN; HEMATOCRIT; and ERYTHROCYTE INDICES. Blood Cell Number,Blood Count, Complete,Blood Cell Counts,Blood Cell Numbers,Blood Counts, Complete,Complete Blood Count,Complete Blood Counts,Count, Blood Cell,Count, Complete Blood,Counts, Blood Cell,Counts, Complete Blood,Number, Blood Cell,Numbers, Blood Cell
D002455 Cell Division The fission of a CELL. It includes CYTOKINESIS, when the CYTOPLASM of a cell is divided, and CELL NUCLEUS DIVISION. M Phase,Cell Division Phase,Cell Divisions,Division Phase, Cell,Division, Cell,Divisions, Cell,M Phases,Phase, Cell Division,Phase, M,Phases, M
D003114 Colony-Forming Units Assay A cytologic technique for measuring the functional capacity of stem cells by assaying their activity. Clonogenic Cell Assay,Stem Cell Assay,Clonogenic Cell Assays,Colony Forming Units Assays,Colony-Forming Units Assays,Stem Cell Assays,Assay, Clonogenic Cell,Assay, Colony-Forming Units,Assay, Stem Cell,Assays, Clonogenic Cell,Assays, Colony-Forming Units,Assays, Stem Cell,Colony Forming Units Assay
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000741 Anemia, Aplastic A form of anemia in which the bone marrow fails to produce adequate numbers of peripheral blood elements. Anemia, Hypoplastic,Aplastic Anaemia,Aplastic Anemia,Anaemia, Aplastic,Aplastic Anaemias,Aplastic Anemias,Hypoplastic Anemia,Hypoplastic Anemias
D012397 Rosette Formation The in vitro formation of clusters consisting of a cell (usually a lymphocyte) surrounded by antigenic cells or antigen-bearing particles (usually erythrocytes, which may or may not be coated with antibody or antibody and complement). The rosette-forming cell may be an antibody-forming cell, a memory cell, a T-cell, a cell bearing surface cytophilic antibodies, or a monocyte possessing Fc receptors. Rosette formation can be used to identify specific populations of these cells. Immunocytoadherence,Formation, Rosette,Formations, Rosette,Immunocytoadherences,Rosette Formations
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