Is Acute Fibrinous and Organizing Pneumonia the Expression of Immune Dysregulation?

Labarinas, Sonia; Gumy-Pause, Fabienne; Rougemont, Anne-Laure; Baerlocher, Gabriela; Leibundgut, Elisabeth O.; Porret, Naomi; Schäppi, Michela G.; Barazzone-Argiroffo, Constance; Passweg, Jakob; Merlini, Laura; Ozsahin, Hulya; Ansari, Marc (2013). Is Acute Fibrinous and Organizing Pneumonia the Expression of Immune Dysregulation? Journal of pediatric hematology, oncology, 35(2), pp. 139-143. Hagerstown, Md.: Lippincott Williams & Wilkins 10.1097/MPH.0b013e31827e5782

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INTRODUCTION: Acute fibrinous and organizing pneumonia (AFOP) is a recently described histologic pattern of diffuse pulmonary disease. In children, all cases reported to date have been fatal. In this study, we describe the first nonfatal AFOP in a child and review the literature. DESCRIPTION: A 10-year-old boy developed very severe aplastic anemia (VSAA) after being admitted to our hospital with a fulminant hepatic failure of unknown origin. A chest computed tomography scan revealed multiple lung nodules and a biopsy of a pulmonary lesion showed all the signs of AFOP. Infectious workup remained negative. We started immunosuppressive therapy with antithymocyte globulin and cyclosporine to treat VSAA. Subsequent chest computed tomography scans showed a considerable diminution of the lung lesions but the VSAA did not improve until we performed hematopoietic stem cell transplantation 5 months later. CONCLUSIONS: Aplastic anemia is associated with a variety of autoimmune syndromes. The sequence of events in our patient suggests that the hepatic failure, AFOP, and the VSAA may all have been part of an autoimmune syndrome. AFOP could be the result of immune dysregulation in this pediatric case with favorable outcome after immunosuppressive therapy and hematopoietic stem cell transplantation.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Haematology, Oncology, Infectious Diseases, Laboratory Medicine and Hospital Pharmacy (DOLS) > Clinic of Haematology and Central Haematological Laboratory

UniBE Contributor:

Baerlocher, Gabriela M.; Oppliger Leibundgut, Elisabeth and Porret, Naomi

Subjects:

600 Technology > 610 Medicine & health

ISSN:

1077-4114

Publisher:

Lippincott Williams & Wilkins

Language:

English

Submitter:

Factscience Import

Date Deposited:

04 Oct 2013 14:32

Last Modified:

26 Nov 2015 10:06

Publisher DOI:

10.1097/MPH.0b013e31827e5782

PubMed ID:

23337545

Web of Science ID:

000315357100024

BORIS DOI:

10.7892/boris.12598

URI:

https://boris.unibe.ch/id/eprint/12598 (FactScience: 218960)

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