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Mycobacterium avium Complex Infection in a Patient with Sickle Cell Disease and Severe Iron Overload.

Shemisa K, Jafferjee N, Thomas D, Jacobs G, Meyerson HJ - Case Rep Infect Dis (2014)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: University of Texas Southwestern University Hospital, 5323 Harry Hines Boulevard, Dallas, TX 75390, USA.

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.

No MeSH data available.


Related in: MedlinePlus

(a) Liver biopsy demonstrating large noncaseating granuloma. (b) Prussian blue staining for iron elements confirms severe hemosiderosis.
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fig2: (a) Liver biopsy demonstrating large noncaseating granuloma. (b) Prussian blue staining for iron elements confirms severe hemosiderosis.

Mentions: The initial investigation included computed tomography (CT) of the chest/abdomen/pelvis which was significant for an enlarged hyperdense liver and left adrenal gland. These features were consistent with hepatic hemosiderosis. (Figure 1). Other findings included multiple lung nodules as well as nonspecific axillary, mediastinal, and periportal lymphadenopathy. She then underwent an uncomplicated liver biopsy which was histopathologically consistent for severe secondary hemosiderosis with noncaseating granulomas (Figure 2). The clinical, radiographic, and pathological findings initially supported the diagnosis for sarcoidosis with underlying iron overload of which systemic corticosteroids (Prednisone 60 mg) were prescribed. An HIV test was ordered prior to initiation of therapy and was nonreactive. There was minimal clinical improvement with steroid treatment and she subsequently developed new pancytopenia.


Mycobacterium avium Complex Infection in a Patient with Sickle Cell Disease and Severe Iron Overload.

Shemisa K, Jafferjee N, Thomas D, Jacobs G, Meyerson HJ - Case Rep Infect Dis (2014)

(a) Liver biopsy demonstrating large noncaseating granuloma. (b) Prussian blue staining for iron elements confirms severe hemosiderosis.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4269307&req=5

fig2: (a) Liver biopsy demonstrating large noncaseating granuloma. (b) Prussian blue staining for iron elements confirms severe hemosiderosis.
Mentions: The initial investigation included computed tomography (CT) of the chest/abdomen/pelvis which was significant for an enlarged hyperdense liver and left adrenal gland. These features were consistent with hepatic hemosiderosis. (Figure 1). Other findings included multiple lung nodules as well as nonspecific axillary, mediastinal, and periportal lymphadenopathy. She then underwent an uncomplicated liver biopsy which was histopathologically consistent for severe secondary hemosiderosis with noncaseating granulomas (Figure 2). The clinical, radiographic, and pathological findings initially supported the diagnosis for sarcoidosis with underlying iron overload of which systemic corticosteroids (Prednisone 60 mg) were prescribed. An HIV test was ordered prior to initiation of therapy and was nonreactive. There was minimal clinical improvement with steroid treatment and she subsequently developed new pancytopenia.

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: University of Texas Southwestern University Hospital, 5323 Harry Hines Boulevard, Dallas, TX 75390, USA.

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.

No MeSH data available.


Related in: MedlinePlus