title: Mycobacterium avium Complex Infection in a Patient with Sickle Cell Disease and Severe Iron Overload.
abstract: A 34-year-old female with sickle cell anemia (hemoglobin SS disease) and severe iron overload presented to our institution with the subacute presentation of recurrent pain crisis, fever of unknown origin, pancytopenia, and weight loss. A CT scan demonstrated both lung and liver nodules concerning for granulomatous disease. Subsequent biopsies of the liver and bone marrow confirmed the presence of noncaseating granulomas and blood cultures isolated Mycobacterium avium complex MAC. Disseminated MAC is considered an opportunistic infection typically diagnosed in the immunocompromised and rarely in immunocompetent patients. An appreciable number of mycobacterial infection cases have been reported in sickle cell disease patients without immune dysfunction. It has been reported that iron overload is known to increase the risk for mycobacterial infection in vitro and in vivo studies. While iron overload is primarily known to cause end organ dysfunction, the clinical relationship with sickle cell disease and disseminated MAC infection has not been reported. Clinical iron overload is a common condition diagnosed in the sub-Saharan African population. High dietary iron, genetic defects in iron trafficking, as well as hemoglobinopathy are believed to be the etiologies for iron overload in this region. Patients with iron overload in this region were 17-fold more likely to die from Mycobacterium tuberculosis. Both experimental and clinical evidence suggest a possible link to iron overload and mycobacterial infections; however larger observational studies are necessary to determine true causality.
authors.affiliation: University of Texas Southwestern University Hospital, 5323 Harry Hines Boulevard, Dallas, TX 75390, USA.
authors.forename: Kamal
authors.identifier: 0000-0001-6704-4954
authors.initials: K
authors.lastname: Shemisa
authors.affiliation: Yale University Hospital, 20 York Street, New Haven, CT 06510, USA.
authors.forename: Nasima
authors.initials: N
authors.lastname: Jafferjee
authors.affiliation: University Hospitals Case Western Reserve University, 11100 Euclid Avenue, Cleveland, OH 44106, USA.
authors.forename: David
authors.initials: D
authors.lastname: Thomas
authors.affiliation: University Hospitals Case Western Reserve University, 11100 Euclid Avenue, Cleveland, OH 44106, USA.
authors.forename: Gretta
authors.initials: G
authors.lastname: Jacobs
authors.affiliation: University Hospitals Case Western Reserve University, 11100 Euclid Avenue, Cleveland, OH 44106, USA.
authors.forename: Howard J
authors.initials: HJ
authors.lastname: Meyerson