Effects of plasma transfusion on hepcidin production in human congenital hypotransferrinemia

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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
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Paola Trombini1, Tiziana Coliva, Elizabeta Nemeth

  • 1Department of Clinical Medicine and Centre for Diagnosis and Therapy of Hemochromatosis, University of Milano-Bicocca, San Gerardo Hospital, Monza, Milan, Italy.

Haematologica
|September 5, 2007
PubMed

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.

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