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paroxysmal cold hemoglobinuria |
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hemoglobinuria /he·mo·glo·bin·uria/ (he″mo-glo″bĭ-nu´re-ah) free hemoglobin in the urine.hemoglobinu´ric
march hemoglobinuria that seen after prolonged exercise. paroxysmal cold hemoglobinuria an autoimmune or postviral disease marked by episodes of hemoglobinemia and hemoglobinuria after exposure to cold, caused by complement-dependent hemolysis due to Donath-Landsteiner antibody. paroxysmal nocturnal hemoglobinuria (PNH) a chronic acquired blood cell abnormality with episodes of intravascular hemolysis and venous thrombosis. toxic hemoglobinuria that caused by ingestion of a poison.
paroxysmal cold hemoglobinuria (PCH), a rare autoimmune disorder characterized by hemolysis and hematuria, associated with exposure to cold. hemoglobinuria [he″mo-glo″bĭ-nu´re-ah] the presence of free hemoglobin in the urine. adj., adj hemoglobinu´ric. march hemoglobinuria hemolysis caused by repeated uncushioned shocks or trauma to some body part, such as in some soldiers on long marches, in marathon runners, and in karate practitioners. paroxysmal cold hemoglobinuria an autoimmune or postviral disease in which there is a biphasic IgG antibody directed against the P blood group antigen. It is marked by episodes of hemoglobinemia and hemoglobinuria after exposure to cold and is detected by the Donath-Landsteiner test. The condition is treated with prednisone and cyclophosphamide and by protection from exposure to cold. paroxysmal nocturnal hemoglobinuria (PNH) an acquired blood cell abnormality with proliferation of abnormal red blood cells (PNH cells) that are readily hemolyzed by complement, and episodes of severe hemolysis and thrombosis, particularly of the hepatic veins. It is detected by the ham test. Treatment is with androgens or prednisone and, during thrombotic episodes, with heparin.
paroxysmal cold hemoglobinuria Hematology A disorder that is: (1) Rarely 'paroxysmal' clinically; (2) Not always precipitated by the cold; and (3) Not always associated with hemoglobinuria; PCH comprises 2-5% of autoimmune
hemolytic anemias, and is caused by IgG–Donath-Landsteiner antibodies that react at < 15ºC and are directed against the ubiquitous P antigen on red cells; PCH may be transient, and 2º to viral exanthemas of childhood Clinical
After exposure to the cold, the Pt may experience myalgia, abdominal cramping, headaches, hemoglobinuria, Raynaud phenomenon, cold urticaria, jaundice Lab Positive direct Coombs test–using anti-C3 antiserum, anemia, hemoglobinuria, ↓
haptoglobin, ↑ LD, ↑ BR; the antibody is a non-agglutinating IgG that binds to RBCs at cold temperatures and, when warmed to 37ºC, evokes complement-mediated hemolyses; antibody elutes from the red cells in vitro, and complement
remains fixed, and thus is a 'biphasic' hemolysin; anti-P reacts with normal neutrophil antigens except for p and Pk Prevention Keep warm Management If chronic, corticosteroids, immunosuppressants Want to thank TFD for its existence? Tell a friend about us, add a link to this page, add the site to iGoogle, or visit the webmaster's page for free fun content. |
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