Relationship between aplastic anemia and paroxysmal nocturnal hemoglobinuria.
Since aplastic anemia-paroxysmal nocturnal hemoglobinuria syndrome was reported in 1967, the overlap of idiopathic aplastic anemia (AA) and paroxysmal nocturnal hemoglobinuria (PNH) has been well known. The link between the 2 diseases became even more evident when immunosuppressive therapy improved survival of patients with severe AA. More than 10% of patients with AA develop clinically evident PNH. Moreover, flow cytometric analysis demonstrates that the majority of patients with AA have a subclinical percentage of granulocytes with PNH phenotype. Some of them have clearly recognizable PNH clones. Granulocytes with a PNH phenotype are also often found in normal individuals, though at much smaller percentages of cells. This finding suggests that a PNH clone is expanded in AA. consistent with a hypothesis that blood cells from patients with PNH are more resistant to an autoimmune environment. Survival of PNH clones in pathologic bone marrow may account for limited expansion of PNH clones; however, additional genetic change(s) that confers cells with growth phenotype may be required for the full development of PNH.
Kinoshita
Taroh
T
Department of Immunoregulation, Research Institute for Microbial Diseases, Osaka University, Suita, Japan.
Inoue
Norimitsu
N
D000741:Anemia, Aplastic / Q000209:etiology*; D002999:Clone Cells / Q000473:pathology; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications* / Q000473:pathology; D006461:Hemolysis; D006801:Humans; D008565:Membrane Proteins / Q000235:genetics
D008565:Membrane Proteins; C080086:phosphatidylinositol glycan-class A protein