To investigate the greater fixation of C3 to the erythrocytes of patients with paroxysmal nocturnal hemoglobinuria (PNH) upon activation of complement, we have examined the formation and the reaction of the C3 nephritic factor-stabilized alternative pathway convertase made with purified components on normal and PNH erythrocytes. Each convertase complex converts four to five times more fluid-pha...

Paroxysmal nocturnal hemoglobinuria (PNH) is a hematologic disorder characterized by complement-mediated hemolysis leading to severe complications, such as life threatening thrombosis. Eculizumab, a humanized anti-C5 monoclonal antibody, has dramatically improved outcomes of patients with PNH. Despite this new revolutionary treatment, clinical information regarding eculizumab use in pregnant wo...

Paroxysmal nocturnal hemoglobinuria (PNH) results from somatic mutations in the PIG-A gene, leading to poor presentation of glycosylphosphatidylinositol (GPI)-anchored surface proteins. PNH frequently occurs in association with suppressed hematopoiesis, including frank aplastic anemia (AA). The relationship between GPI-anchored protein expression and bone marrow (BM) failure is unknown. To asse...

I N 1961, WE REPORTED 20 cases of severe aplastic anemia in which infusions of allogenic ( homologous ) bone marrow had been used as one of the therapeutic methods.1 Seven of these patients made apparently complete recoveries; whether coincidentally or in rlationship to the marrow infusions is not clear. Since then, the use of allogeneic bone marrow infusions has been wellnigh discarded for the...

I N 1961, WE REPORTED 20 cases of severe aplastic anemia in which infusions of allogenic ( homologous ) bone marrow had been used as one of the therapeutic methods.1 Seven of these patients made apparently complete recoveries; whether coincidentally or in rlationship to the marrow infusions is not clear. Since then, the use of allogeneic bone marrow infusions has been wellnigh discarded for the...

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal disorder of the hematopoietic stem cell (HSC). Somatic mutations in the PIG-A gene result in the deficiency of several glycosylphosphatidylinositol-linked proteins from the surface of blood cells. This explains intravascular hemolysis but does not explain the mechanism of bone marrow failure that is almost invariably seen in PNH. I...

BACKGROUND In 2015, 5.3 million babies died in the third trimester of pregnancy and first month following birth. Progress in reducing neonatal mortality and stillbirth rates has lagged behind the substantial progress in reducing postneonatal and maternal mortality rates. The benefits to prenatal and neonatal health (PNH) from maternal and child health investments cannot be assumed. METHODS We...

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia in which venous thrombosis is the most common cause of death. Here we address the risk factors for thrombosis and the role of warfarin prophylaxis in PNH. The median follow-up of 163 PNH patients was 6 years (range, 0.2-38 years). Of the patients, 29 suffered thromboses, with a 10-year incidence of 23%. There were 9 patie...

The association of paroxysmal nocturnal hemoglobinuria (PNH) and aplastic anemia (AA) raises the yet unresolved questions as to whether these two disorders are different forms of the same disease. We compared two groups of patients with respect to cytogenetic features, glycosylphosphatidylinositol (GPII-linked protein expression, protein C/ protein Slthrombomodulinlantithrombin 111 activity, an...

The association of paroxysmal nocturnal hemoglobinuria (PNH) and aplastic anemia (AA) raises the yet unresolved questions as to whether these two disorders are different forms of the same disease. We compared two groups of patients with respect to cytogenetic features, glycosylphosphatidylinositol (GPII-linked protein expression, protein C/ protein Slthrombomodulinlantithrombin 111 activity, an...