Paroxysmal nocturnal hemoglobinuria

摘要: Abstract Markedly reduced erythrocyte acetylcholinesterase activity was demonstrated in all eight cases of paroxysmal nocturnal hemoglobinuria studied. Consistent reduction this degree has not been noted other blood dyscrasias, either studies cited the literature or our own investigations. The defect localized to stroma PNH red cell. enzyme reticulocytes markedly impaired, indicating that immature cells were formed with defective acetylcholinesterase. normal following transfusion into patients PNH. No evidence found for a circulating inhibitor one which could be readily eluted from membrane. However, do exclude possibility an inhibitor, particularly bound firmly enzyme. In case family study carried out, present members tested. previous work many investigators suggested plays important role preservation integrity significance cell is discussed connection.