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Abstract
Background Accurate assessment of iron status is important in patients with acute hepatic porphyrias (AHP) since iron overload may develop after repeated administration of hemin. Serum ferritin is a useful measure of iron status; however, ferritin is also an acute-phase protein that may be elevated by inflammation or dysmetabolic conditions. We considered that ferritin elevation after hemin administration could result from an acute phase response in AHP rather than sustained iron overload. We examined data from five patients with acute intermittent porphyria (AIP) who were treated with hemin and had multiple ferritin measurements, providing an opportunity to assess time relationships between hemin administration and serum ferritin.
Study findings The dose-time relationship between hemin administration and serum ferritin for each patient is shown in the figure 1. Patients 1 and 2 received prophylactic and treatment doses of hemin, while patients 3,4, and 5 received prophylactic givosiran and treatment doses of hemin. All patients had normal baseline serum ferritin levels, increase in ferritin post-hemin, and a decline in ferritin levels over time. Sustained ferritin elevations were not noted even after repeated hemin administration. Also, serum ferritin rise post-hemin administration varied widely – hemin given to a patient in iron-deficient state did not raise ferritin as dramatically as when it was given in iron-replete state.
Conclusion Our observations suggest that serum ferritin increases rapidly after hemin administration and decreases slowly over several days or weeks, and during that time may not be a reliable indicator of iron overload. Prospective, larger studies are needed to assess the time course of ferritin elevation after hemin administration, relationship with serum iron status, and measurement of hepatic iron concentrations in order to determine risk for iron overload and hepatic damage in AHPs.
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