Neutrophil life span in paroxysmal nocturnal hemoglobinuria.

The life-span of complement-sensitive and -insensitive red cells in paroxysmal nocturnal hemoglobinuria.

Intestinal perforation in a patient with paroxysmal nocturnal hemoglobinuria

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder that manifests with hemolytic anemia, thrombosis, and peripheral blood cytopenias.Acute abdominal pain is one of the PNH clinical manifestations due to venous thrombosis of intra-abdominal sites including hepatic, portal, mesenteric, and splenic veins.Eculizumaband allogeneic bone marrow transplantation (BMT) arethe only w...

Life Span of Reticulocytes in Paroxysmal Nocturnal Hemoglobinuria.

P REVIOUS OBSERVATIONS have demonstrated the shortened life span of the red cell in patients with paroxysmal nocturnal hemoglohinuria ( PNH ).1.2 Studies of the survival of homologous PNH red cells by the Ashby technic and of autologous PNH cells labeled with radioactive sodium chromate have demonstrated a short and biphasic red cell survival curve in the patient and normal recipient. This life...

Loss of expression of neutrophil proteinase-3: a factor contributing to thrombotic risk in paroxysmal nocturnal hemoglobinuria.

BACKGROUND A deficiency of specific glycosylphosphatidyl inositol-anchored proteins in paroxysmal nocturnal hemoglobinuria may be responsible for most of the clinical features of this disease, but some functional consequences may be indirect. For example, the absence of certain glycosylphosphatidyl inositol-anchored proteins in paroxysmal nocturnal hemoglobinuria cells may influence expression ...

Baseline characteristics and disease burden in patients in the International Paroxysmal Nocturnal Hemoglobinuria Registry.

Paroxysmal nocturnal hemoglobinuria is a rare, acquired disease associated with hemolytic anemia, bone marrow failure, thrombosis, and, frequently, poor quality of life. The International PNH Registry is a worldwide, observational, non-interventional study collecting safety, effectiveness, and quality-of-life data from patients with a confirmed paroxysmal nocturnal hemoglobinuria diagnosis or d...