Flow cytometry is the gold standard in diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) by detecting the absence of glycol-phosphatidyl inositol (GPI)-linked protein expression on granulocyte and monocyte surfaces. However, the current assays are not optimized and require improvement, particularly in reducing background fluorescence and optimizing sensitivity and specificity. With more fl...

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired disorder in which erythrocytes, granulocytes, and platelets are defective, as shown by increased susceptibility of RBCs, WBCs, and platelets to complement-mediated lysis in vitro. The purpose of this study is to determine the sensitivity to complement lysis of PNH and non-PNH erythroid and myeloid precursors using the release of 59Fe and ...

T AROXYSMAL NOCTURNAL HEMOGLOBINURIA (PNH) is a relaI tively rare chronic hemolytic anemia, but its clinical and laboratory characteristics have been well covered in the recent literature. Crosby’ has published a comprehensive review of the literature on this subject, and Dacie2 has summarized much of the information available on the mechanism of hemolysis in this disea se. It is recognized tha...

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 a rare hematological disorder associated with an acquired deficiency in glycophosphatidylinositol-anchor biosynthesis that renders erythrocytes susceptible to complement attack. Intravascular hemolysis via the membrane attack complex is a clinical hallmark of the disease, and C5 blockade is currently the only approved treatment for PNH. However, resi...

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare bone marrow failure disorder that manifests with hemolytic anemia, thrombosis, and peripheral blood cytopenias. The absence of two glycosylphosphatidylinositol (GPI)-anchored proteins, CD55 and CD59, leads to uncontrolled complement activation that accounts for hemolysis and other PNH manifestations. GPI anchor protein deficiency is almost alw...

Paroxysmal nocturnal hemoglobinuria (PNH) is a disease characterized by chronic persistent hemolysis, multi-organ damage and eventually multiple organ failure. PNH develops as a result of increased sensitivity to complement due to an acquired deficiency of certain glycosylphosphatidylinositol (GPI)-linked proteins. The clinical presentation of PNH varies greatly from one patient to another. We ...

Studies of children with paroxysmal nocturnal hemo-globinuria (PNH) are scarce and include limited numbers of patients. We compared demographic and clinical characteristics of children and adults in the International PNH Registry. Our data show that, compared to adults with PNH, children had smaller PNH clones with more frequent severe cytopenia or previous marrow aplasia. In contrast, hemolysi...