" /> Non-HFE-related hereditary haemochromatosis, Type 4 - CISMeF





Preferred Label : Non-HFE-related hereditary haemochromatosis, Type 4;

ICD-11 definition : Hemochromatosis type 4 (also called ferroportin disease) is a rare form of hereditary hemochromatosis, a group of diseases characterized by excessive tissue iron deposition of genetic origin. It is phenotypically heterogeneous with two sub-types. Ferroportin disease form A is the usual form and is generally asymptomatic with no tissue damage and further complications. Ferroportin disease form B is rarer and resembles hemochromatosis type 1, but can affect children.;

ICD-11 synonym : Ferroportin disease; Haemochromatosis due to defect in ferroportin; Autosomal dominant hereditary haemochromatosis;

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Hemochromatosis type 4 (also called ferroportin disease) is a rare form of hereditary hemochromatosis, a group of diseases characterized by excessive tissue iron deposition of genetic origin. It is phenotypically heterogeneous with two sub-types. Ferroportin disease form A is the usual form and is generally asymptomatic with no tissue damage and further complications. Ferroportin disease form B is rarer and resembles hemochromatosis type 1, but can affect children.

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08/07/2025


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