paroxysmal cold hemoglobinuria

hemoglobinuria /he·mo·glo·bin·uria/ (he″mo-glo″bĭ-nu´re-ah) free hemoglobin in the urine.hemoglobinu´ric

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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.

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paroxysmal cold hemoglobinuria (PCH),

a rare autoimmune disorder characterized by hemolysis and hematuria, associated with exposure to cold.

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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