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

Labarinas, Sonia MD; Gumy-Pause, Fabienne MD; Rougemont, Anne-Laure MD; Baerlocher, Gabriela MD; Leibundgut, Elisabeth O. MD; Porret, Naomi MD; Schäppi, Michela G. PD; Barazzone-Argiroffo, Constance PD; Passweg, Jakob MD; Merlini, Laura MD; Ozsahin, Hulya PD; Ansari, Marc MD

Journal of Pediatric Hematology/Oncology:
doi: 10.1097/MPH.0b013e31827e5782
Original Articles
Abstract

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.

Author Information

*Department of Pediatrics, Hemato-oncology unit

Division of Clinical Pathology

Geneva University Hospital

§Department of Clinical Research, Experimental Hematology, Gastroenterology unit, University of Bern, Bern

Pneumology unit

Department of Internal Medicine, Hematology service

#Department of Hematology, Radiology Service, University Hospital of Geneva, Geneva, Switzerland

M.A. and S.L.: Designed the research and the article, drafted the article, and performed the analysis; A.L.R., G.M.B., E.O.; N.A.P.; and L.M.: Performed experiments; M.A., S.L., and L.M.: Contributed to the retrieval of clinical data; all authors contributed to the interpretation of data and revised the manuscript critically.

M.A. is scholar of the Telemaque foundation. The remaining authors declare no conflict of interest.

Reprints: Sonia Labarinas, MD, University Hospital of Geneva, rue Willy Donzé 6, CH-1211 Geneva 14, Switzerland (e-mail: sonia.labarinas@hcuge.ch).

Received November 16, 2011

Accepted November 15, 2012

Copyright © 2013 Wolters Kluwer Health, Inc. All rights reserved.