title: Fetal hemoglobin in paroxysmal nocturnal hemoglobinuria (PNH): evidence for derivation of HbF-containing erythrocytes (F cells) from the PNH clone as well as from normal hemopoietic stem cell lines.
abstract: The cellular distribution of HbF was studied in nine patients with paroxysmal nocturnal hemoglobinuria (PNH) by measuring the level of HbF and determining the number of HbF-containing red cells (F cells) in whole blood and in the population of normal cells obtained after immune lysis of the abnormal erythrocytes. The amounts of HbF and the F cell frequencies found in the normal red cells were strikingly similar to the values seen in whole blood. The observed frequencies of F cells in normal cells best fitted those expected under the assumption that the F cells arise equally from normal hemopoietic stem cells and from the stem cells with the PNH defect. Since PNH appears to be a clonal hemopoietic stem cell disorder, this evidence argues against a derivation of F cells from distinct pluripotent stem cell lines.
mesh_terms: D002460:Cell Line; D002469:Cell Separation; D002999:Clone Cells; D004912:Erythrocytes; D005319:Fetal Hemoglobin; D005455:Fluorescent Antibody Technique; D006412:Hematopoietic Stem Cells; D006457:Hemoglobinuria, Paroxysmal / Q000097:blood*; D006461:Hemolysis; D006801:Humans
chemical_list: D005319:Fetal Hemoglobin