Genetic and environmental effects in paroxysmal nocturnal hemoglobinuria: this little PIG-A goes “Why? Why? Why?”

Abstract excerpt

Paroxysmal nocturnal hemoglobinuria (PNH) has been a medical curiosity since the description of the first case in the mid–19th century by the English physician Sir William Gull. (See Rosse [ref. 1] for an eloquent history of studies on this disease.) Gull’s patient suffered morning crises of “intermittent hematinuria,” which was later recognized as the destruction of red cells in circulation. Intravascular...

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