title: Dacie JV. Diagnosis and mechanism of hemolysis in chronic hemolytic anemia with nocturnal hemoglobinuria.
abstract: A leading British hematologist, John V. Dacie was known for his pioneering work on hemolytic disorders, especially paroxysmal nocturnal hemoglobinuria (PNH). In 1949, 12 years after the description of the Ham test for PNH, Dacie commented that “the essential basis of the disease is still a mystery.” In this article, he demonstrated that the Ham test is highly dependent on acidification and requires a serum factor destroyed by heating. He concluded that PNH is a cell-intrinsic defect of erythrocytes that reflects a cell surface abnormality and requires heat-labile serum factors. More than 40 years later, Kinoshita and colleagues reported that the defective surface expression of the glycosylphosphatidylinositol-anchored proteins is caused by a somatic mutation of the PIG-A gene [Takeda J et al, Cell. 1993;73(4):703-711].
mesh_terms: D000743:Anemia, Hemolytic / Q000175:diagnosis / Q000473:pathology; D016923:Cell Death; D002908:Chronic Disease; D004912:Erythrocytes / Q000473:pathology*; D006403:Hematologic Tests; D006457:Hemoglobinuria, Paroxysmal / Q000175:diagnosis / Q000473:pathology*; D006461:Hemolysis; D006801:Humans