Causes of death in hyper-IgE syndrome

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Presentation transcript:

Causes of death in hyper-IgE syndrome Alexandra F. Freeman, MD, David E. Kleiner, MD, Hari Nadiminti, MD, Joie Davis, APRN, APNG, Martha Quezado, MD, Victoria Anderson, MSN, CRNP, Jennifer M. Puck, MD, Steven M. Holland, MD Journal of Allergy and Clinical Immunology Volume 119, Issue 5, Pages 1234-1240 (May 2007) DOI: 10.1016/j.jaci.2006.12.666 Copyright © 2007 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 1 Chest CT of patient 1 showing pneumatocoeles characteristic of HIES, with aspergilloma present within the pneumatocoele. Journal of Allergy and Clinical Immunology 2007 119, 1234-1240DOI: (10.1016/j.jaci.2006.12.666) Copyright © 2007 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 2 A, Patient 3, gross lung specimen showing pneumatoceles. B, Patient 6, fungal elements within bronchus. C, Patient 6, fungal elements within pulmonary cavity (GMS stain). D, Patient 6, pneumocystis adjacent to the pulmonary cavity (GMS stain). E, Patient 5, vessel with infected thrombus. F, Patient 2, fungal elements within bronchiole with granulomatous inflammation. Journal of Allergy and Clinical Immunology 2007 119, 1234-1240DOI: (10.1016/j.jaci.2006.12.666) Copyright © 2007 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 3 A, Patient 2, cerebritis with budding hyphae (GMS stain). B, Patient 5, left middle cerebral artery thombosis with fungal elements (GMS stain). Journal of Allergy and Clinical Immunology 2007 119, 1234-1240DOI: (10.1016/j.jaci.2006.12.666) Copyright © 2007 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 4 Angiomyolipoma found in renal cortex of patient 3. A, Interface between tumor and normal kidney. A normal glomerulus is indicated by the arrow. The tumor is composed of fats and eosinophilic spindle cells. Trapped tubules are present within the tumor (hematoxylin and eosin stain, ×200). B, Immunohistochemical staining for smooth muscle actin, which is strongly positive in the spindle cell component of the tumor but negative in renal tubular cells and glomeruli (anti–smooth muscle actin stain, ×200). Journal of Allergy and Clinical Immunology 2007 119, 1234-1240DOI: (10.1016/j.jaci.2006.12.666) Copyright © 2007 American Academy of Allergy, Asthma & Immunology Terms and Conditions