Paroxysmal nocturnal hemoglobinuria; plasma factors of the hemolytic system.
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منابع مشابه
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...
متن کاملParoxysmal nocturnal hemoglobinuria: the role of properdin in the diagnostic thrombin test.
By WILLIArsI H. CROSBY AND NAOMI R. BENJAMIN I HE RED BLOOD CELLS of patients with paroxysmal nocturnal hemoglobinuria ( PNH ) possess an apparently unique susceptibility to the hemolytic activity of certain naturally occurring plasma factors. Hemolysis occurs readily in vitro and the reaction is intensified when the incubating mixture has been made slightly acid. This phenomenon is the basis o...
متن کاملDecreased hemolytic capacity of sera form patients with paroxysmal nocturnal hemoglobinuria.
T HE ERYTHROCYTES of patients with Paroxysmal Nocturnal Hemoglobinuria (PNH) hemolyse at 37 C. in normal compatible human sera in the absence of demonstrable antibody. The reaction is complement dependent and in vivo causes a hemolytic anemia of varying severity. The diagnostic acid hemolysis test for this disorder is performed by demonstrating hemolysis of suspect erythrocytes in the patient’s...
متن کاملParoxysmal Nocturnal Hemoglobinuria : The Role of Antibodies in the Diagnostic Thrombin Test
N ABNORMALITY of the red cells of paroxysmal nocturnal hemoglobinuria ( PNH ) is responsible for their short life span in the circulation anu their lysis in vitro by certain factors present in normal plasma or serum. The hemolytic system in vitro is characteristically more active when the serum is made somewhat acid, about pH 6.8. The addition of thrombin to the serum is also capable of increas...
متن کاملSmall-molecule factor D inhibitors selectively block the alternative pathway of complement in paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome
Paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome are diseases of excess activation of the alternative pathway of complement that are treated with eculizumab, a humanized monoclonal antibody against the terminal complement component C5. Eculizumab must be administered intravenously, and moreover some patients with paroxysmal nocturnal hemoglobinuria on eculizumab have s...
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عنوان ژورنال:
- Blood
دوره 8 5 شماره
صفحات -
تاریخ انتشار 1953