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Paroxysmal cold hemoglobinuria is an autoimmune hemolytic anemia featured by complement-mediated intravascular hemolysis after cold exposure. It can present as an acute non-recurrent postinfectious event in children, or chronic relapsing episodes in adults with hematological malignancies or tertiary syphilis. Described by Julius Donath and Karl Landsteiner in 1904, PCH is one of the first clinical entities recognized as an autoimmune disorder.
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