Table 1.

Hepcidin regulation and dysregulation in physiological and pathological conditions

Causation	Disease/condition	Pathophysiology	Serum/plasma hepcidin
Hepcidin changes alter iron homeostasis	Iron-refractory iron deficiency anemia (TMPRSS6 mutations)	Genetic overproduction of hepcidin causes iron restriction	High normal to high
	Infections, rheumatologic diseases, inflammatory bowel disease, cancer	Inflammation increases hepcidin synthesis, resulting in iron restriction	High
	Chronic kidney disease	Inflammation and decreased renal clearance of hepcidin results in hepcidin excess and iron restriction; iron therapy may raise hepcidin further	High
	Hereditary hemochromatosis (HFE, TFR2, HJV, HAMP mutations)	Genetic hepcidin deficiency causes iron overload	Undetectable, low, or low for iron load
	Non-transfusion-dependent thalassemia	Ineffective erythropoiesis and high erythropoietic drive stimulate erythroferrone production, suppressing hepcidin and resulting in iron overload	Low
	Hepatitis C, alcoholic liver disease	Suppression of hepcidin by alcohol, virus, growth factors, and loss of hepatocytes results in iron loading	Low
	Pregnancy	Pregnancy-related hepcidin-suppressive factor results in iron mobilization	Low
Hepcidin changes appropriately reflect iron physiology	Iron deficiency	Blood loss, malnutrition	Low/undetectable
	Secondary iron overload	Transfusions, iron therapy	High
	Hereditary hemochromatosis (SLC40A1 mutations)	Ferroportin resistance to hepcidin causes iron overload	High
Mixed disorders	Transfusion-dependent thalassemia	Ineffective erythropoiesis suppresses hepcidin, and transfusional iron overload increases hepcidin	Relatively low for iron load, fluctuates during the transfusion cycle