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Effects of plasma transfusion on hepcidin production in human congenital hypotransferrinemia

From Department of Clinical Medicine and Centre for Diagnosis and Therapy of Hemochromatosis, University of Milano-Bicocca, San Gerardo Hospital, Monza (Milan), Italy. (PT, RM, AP); Department of Pediatrics, University of Milano-Bicocca, San Gerardo Hospital, Monza, Italy (TC, AB); David Geffen School of Medicine, University of California, Los Angeles, USA (EN, TG)

Correspondence: Alberto Piperno, Division of Clinical Medicine, University of Milano-Bicocca, San Gerardo Hospital, Via Pergolesi 33, 20052 Monza, Italy. E-mail: alberto.piperno{at}unimib.it

Hepcidin is the key regulator of systemic iron homeostasis. We describe the modulation of hepcidin production induced by plasma transfusions in a patient with congenital hypotransferrinemia that offers a unique model in which to study the mechanism of hepcidin regulation by iron and erythropoiesis. Urinary hepcidin increased from zero at baseline, when hemoglobin and serum transferrin was low, to a maximum of 98 ng/mg creatinine on day 60, and subsequently decreased. Time-course of urinary hepcidin and serum transferrin concentration suggests that hepcidin production is regulated by the combination of marrow iron requirements and iron supply by transferrin.

Key words: hypotransferrinemia, hepcidin, transferrin saturation, transfusion, eryhtropoiesis.