Issn Print: 1056-9103

Publication Date: 2001/01/01


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Hemochromatosis as a Risk Factor for Fulminant Staphylococcus aureus Pneumonia*

Darrell J. Baranko; Joseph M. Flynn; Jeremy R. Blanchard

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Excerpt

Hemochromatosis is a disease of systemic iron overload characterized by iron deposition in various organs, including the liver, heart, and pancreas, which ultimately leads to end-organ dysfunction and failure if not adequately treated. A less commonly recognized consequence of systemic iron overload is its effect on limiting the ability to fight bacterial infection through its support of bacterial growth and its inhibition of normal immune function. We present a case of a 36-year-old white male who developed Staphylococcus aureus pneumonia, which subsequently lead to his death. At autopsy, he was found to have hemochromatosis. This case demonstrates the clinical effect of iron overload on infection. It illustrates the need for early identification of hemochromatosis patients, because their immunocompromised state warrants a more aggressive approach to infection. Also, an understanding of the underlying pathophysiology of iron overload points to new antimicrobial strategies including new antibiotic design and new transfusion strategies.
Hemochromatosis is a disease of systemic iron overload characterized by iron deposition in multiple organs. In genetic hemochromatosis, there is an excessive absorption of dietary iron in the small bowel. The mechanism for this physiologic iron hoarding is unknown. The excess iron is then stored in parenchymal cells throughout the body. The accumulation of these iron stores causes organ damage over time reflected in the more common clinical presentations. The classic clinical triad is diabetes, bronze skin coloration, and cirrhosis. Other common clinical effects of the excessive body iron overload are arthritis, cardiomyopathy, and pituitary-related hypogonadism. Clinically, this disease is more commonly expressed in men, and is initially diagnosed most often in the 40-to 60-year-old patient [1].
Beyond these commonly recognized abnormalities associated with iron overload states, including hemochromatosis, is the effect on limiting the ability to fight bacterial infection. It is known that bacteria require iron for growth and survival, and iron overload states have been shown to negatively affect the immune system at various levels [2,3,4,5]. This could be the reason behind the increased susceptibility to infection with Vibrio vulnificus, Listeria monocytogenes, Yersinia enterocolitica, Salmonella enteritidis serotype typhimurium, Klebsiella pneumoniae, Escherichia coli, Rhizopus arrhizus, and mucor species that is found in patients with hemochromatosis [1]. We present a case of a 36-year-old white male who developed Staphylococcus aureus (S. aureus) pneumonia, which subsequently lead to his death. At autopsy, he was found to have hemochromatosis. Our review of the literature has revealed several specific mechanisms by which this patient’s underlying state of iron overload contributed to the severity of his infection and, ultimately, to his death. Early identification of hemochromatosis patients is critical not only for prevention of chronic sequelae of the disease, but also for prevention of acute infectious complications that may be lessened in severity by a more aggressive surveillance. Recognition of the underlying mechanisms of increased susceptibility to infection opens the door for novel therapeutic strategies.

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