Introduction

Primary iron overload is a problem among African Americans, but the condition is not often recognized by clinicians. The prevalence is not known, and whether a genetic defect is responsible is uncertain. In contrast, hereditary iron overload in the form of HLA-linked hemochromatosis is a well-recognized condition among whites in the United States, and a mutation in the newly described HFE gene on chromosome 6 is responsible for most cases. Several explanations for primary iron overload in African Americans are possible: (i) an admixture of the genetic defect for HLA-linked hemochromatosis from the Caucasian population of northern European extraction into the African–American population via interracial mating; (ii) a distinct but unidentified inherited defect that may be implicated in iron overload in Africa; and (iii) a sporadic occurrence of iron overload due to unidentified mechanisms. The HFE defect is rare in the African–American population and has not been identified in most African Americans with iron overload investigated. These observations suggest the possibility that primary iron overload in African Americans may be related to the iron-loading disorder found in the people of sub-Saharan Africa. Iron overload is a common condition in Africa where, in many areas, the prevalence of excessive body iron of a severity to cause damage to the liver is 10% or more.