Paroxysmal nocturnal hemoglobinuria; a specific test for the disease based on the ability of thrombin to activate the hemolytic factor.
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Paroxysmal Nocturnal Hemoglobinuria a Specific Test for the Disease Based on the Ability of Thrombin to Activate the Hemolytic Factor
By WILLIAM H. CROSBY, MAJOR, MC, AUS P AROXYSMAL nocturnal hemoglobinuria (Marchiafava-Micheli syndrome) is a rare form of chronic hemolytic anemia characterized by the presence of abnormal erythrocytes which are susceptible to lysis by a factor normally present in plasma or serum. It has recently been suggested that this factor resembles closely or may even be identical with the coagulation ac...
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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 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 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...
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ورودعنوان ژورنال:
- Blood
دوره 5 9 شماره
صفحات -
تاریخ انتشار 1950