Hemochromatosis Linked to Death From Yersinia Exposure
Researchers with undiagnosed hemochromatosis may be at risk for fatal Y. pestis infection
WEDNESDAY, June 29 (HealthDay News) -- Attenuated strains of virulent laboratory organisms like Yersinia pestis (Y. pestis) may pose a risk to researchers with undiagnosed hemochromatosis, according to a letter published in the June 30 issue of the New England Journal of Medicine.
Karen M. Frank, M.D., Ph.D., from the University of Chicago, and colleagues reported the complete follow-up of a lethal septicemic plague death case. A 60-year-old male researcher with a history of insulin-dependent diabetes, hypertension, and hyperlipidemia was admitted to the emergency department after being ill for a week with increasing shortness of breath, dry cough, fever, chills, and weakness. His condition quickly deteriorated and, despite resuscitation efforts, he died after 13 hours. Serum samples were analyzed, blood was cultured, and an autopsy was performed to identify the cause of death.
The investigators identified Y.pestis in the patient's blood cultures, and the autopsy showed abnormally high levels of iron deposits in the non-cirrhotic liver. Antemortem serum samples showed highly elevated levels of ferritin, iron, total iron-binding capacity, and iron saturation. The high iron level in the liver was indicative of hereditary hemochromatosis, which was confirmed by the presence of a C282Y mutation. Further analysis revealed that the patient had been working with attenuated, nonpigmented Y. pestis isolate UC91309, which was genetically altered with the insertion of an antibiotic resistance cassette. The entry route of the Y. pestis could not be determined.
"Given the probable role of undiagnosed hemochromatosis in this fatal case, researchers working with yersinia species may choose to determine whether or not they have the hemochromatosis mutation," the authors wrote.
One author disclosed a financial relationship with Novartis Vaccines and Diagnostics Inc.