The number of microscopically visible lesions produced on the membrane for a given degree of lysis on normal cells as on PNH cells. Since complement lesions were not formed until C8 or C9 was incorporated into the complement sequence, the results suggest that increased lysis of red cells in PNH is due at least in part to more efficient penetration of the PNH membrane by the terminal lytic seque...

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare chronic hemolytic anemia characterized by an acquired defect of the erythrocyte which renders it susceptible to hemolysis by normal human serum. Previous reports have indicated that magnesium and factors resembling the components of complement are required for the hemolysis in vitro of PNH erythrocytes by normal serum (1, 2). Recently a natura...

T HE OCCURENCE of impaired marrow function in paroxysmal nocturnal hemoglobinuria (PNH) has long been recognized. A smaller group of patients, with an initial diagnosis of aplastic anemia, have been noted to develop signs of PNH during the course of their illness.1 Such persons have been said to have “the aplastic anemia-paroxysmal nocturnal hemoglobinuria syndrome.”2 Also evident, in some case...

Paroxysmal nocturnal hemoglobinuria (PNH) is characterized by the presence in the patient's hematopoietic system of a large cell population with a mutation in the X-linked PIG-A gene. Although this abnormal cell population is often found to be monoclonal, it is not unusual that 2 or even several PIG-A mutant clones coexist in the same patient. Therefore, it has been suggested that the PIG-A gen...

Peripheral nerve hyperexcitability (PNH) is one of the distal peripheral neuropathy phenotypes often present in patients affected by type 2 diabetes mellitus (T2DM). Through in vivo and ex vivo electrophysiological recordings in db/db mice, a model of T2DM, we observed that, in addition to reduced nerve conduction velocity, db/db mice also develop PNH. By using pharmacological inhibitors, we de...

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disease characterized by a clone of blood cells lacking glycosyl phosphatidylinositol (GPI)-anchored proteins at the cell membrane. Deficiency of the GPI-anchored complement inhibitors CD55 and CD59 on erythrocytes leads to intravascular hemolysis upon complement activation. Apart from hemolysis, another prominent feature is a highly ...

In this study, we examined the usefulness of various markers on blood cell populations in the diagnosis of paroxysmal nocturnal hemoglobinuria (PNH). We also evaluated bone marrow specimens, which generally are considered less suitable than blood owing to variable expression of glycosyl phosphatidylinositol (GPI)-linked antigens during hematopoietic cell differentiation. All 15 patients in our ...

To identify candidate antigens in aplastic anemia (AA), we screened proteins derived from a leukemia cell line with serum of an AA patient and identified diazepam-binding inhibitor-related protein 1 (DRS-1). Enzyme-linked immunosorbent assay (ELISA) revealed high titers of anti-DRS-1 antibodies (DRS-1 Abs) in 27 (38.0%) of 71 AA patients displaying increased paroxysmal nocturnal hemoglobinuria ...

I N PAROXYSMAL NOCTURNAL HEMOGLOBINURIA (PNH) accelerated destruction of abnormal red cells occurs within an apparently benign extracorpuscular environment.1 Although the underlying cellular defect has not yet been fully elucidated, it has long been recognized that, in vitro, PNII erythrocytes are extraordinarily susceptible to immune lysis. The possibility exists that, in vivo, destruction of ...

Paroxysmal nocturnal hemoglobinuria is a clonal hematopoietic stem cell disease that manifests with intravascular hemolysis, bone marrow failure, thrombosis, and smooth muscle dystonias. The disease can arise de novo or in the setting of acquired aplastic anemia. All PNH patients to date have been shown to harbor PIG-A mutations; the product of this gene is required for the synthesis of glycosy...