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Hemin (trade name Panhematin) is a sterile, lyophilized form of protoporphyrin IX iron (III) chloride, derived from processed red blood cells. It is indicated for the treatment of recurrent attacks of acute intermittent porphyria (AIP) related to the menstrual cycle, as well as other acute porphyrias like variegate porphyria and hereditary coproporphyria. The drug's primary mechanism of action involves the exogenous replacement of heme, which triggers negative feedback inhibition of delta-aminolevulinic acid (ALA) synthase, the rate-limiting enzyme in the heme biosynthetic pathway. By suppressing this enzyme, hemin reduces the overproduction and systemic accumulation of neurotoxic porphyrin precursors, specifically ALA and porphobilinogen (PBG). Approved by the FDA in 1983, it was the first orphan drug to reach the market in the United States.
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