Iron overload syndromes other than hereditary hemochromatosis
- Stanley L Schrier, MD
Stanley L Schrier, MD
- Editor-in-Chief — Hematology
- Section Editor — Myeloproliferative Disorders; Red Cell Disorders
- Professor of Medicine
- Stanford University School of Medicine
- Bruce R Bacon, MD
Bruce R Bacon, MD
- Professor of Internal Medicine
- Saint Louis University School of Medicine
In normal subjects there is no mechanism to regulate iron loss from the body, which averages about 1 mg/day in adult men from sweat, shed skin cells, and gastrointestinal losses. Premenopausal adult women lose an additional 0.5 to 1.0 mg/day because of menses. Therefore, to insure normal stores of iron within the body, iron absorption must be tightly regulated. A simple calculation will make this clear. If iron absorption is increased in an individual by as little as 1.5 mg/day above the amount needed to achieve homeostasis, this will result in the accumulation of 5.5 grams of iron every decade, 16 grams in 30 years and 33 grams in 60 years. This latter figure corresponds to the amount of iron (30 to 40 g) usually found in patients with clinically detected hereditary hemochromatosis (HH) and explains both the delayed time for the clinical appearance of this disease in men and its rarity in premenopausal women. (See "Regulation of iron balance".)
As a result of the inability to increase iron loss, iron overload is an inevitable response to increased iron entry into the body. This can occur by one of three mechanisms (table 1):
●A massive increase in iron intake
●An increase in iron absorption when iron intake is normal
●The parenteral administration of iron, as with transfusional overload
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- ANEMIA DUE TO INEFFECTIVE ERYTHROPOIESIS
- Mechanism for increased iron absorption
- PORPHYRIA CUTANEA TARDA
- Liver disease
- HFE gene mutations
- HCV infection
- LIVER IRON OVERLOAD WITH NORMAL TRANSFERRIN SATURATION
- AFRICAN IRON OVERLOAD
- TRANSFUSIONAL IRON OVERLOAD
- REPEATED PARENTERAL IRON INFUSION
- Intravenous iron preparations
- Intravenous hematin/hemin
- NEONATAL OR PERINATAL IRON OVERLOAD
- INSULIN RESISTANCE
- EXTREMELY HIGH FERRITIN LEVELS
- CHELATION THERAPY