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

• DOI: 10.1097/mph.0b013e31827e5782
• Publication Date: 2013-01-18T08:11:12Z
• Authors: Sonia Labarinas; Fabienne Gumy-Pause; Anne-Laure Rougemont; Gabriela Baerlocher; Elisabeth O. Leib...; Naomi Porret; Michela G. Schäppi; Constance Barazzo...; Jakob Passweg; Laura Merlini; Hulya Ozsahin; Marc Ansari
• Keywords: Immune Dysregulation; Fulminant

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. this study, we describe the first nonfatal AFOP in child review literature.A 10-year-old boy developed very severe aplastic anemia (VSAA) after being admitted our hospital with fulminant hepatic failure unknown origin. A chest computed tomography scan revealed multiple lung nodules biopsy lesion showed signs AFOP. Infectious workup remained negative. We started immunosuppressive therapy antithymocyte globulin cyclosporine treat VSAA. Subsequent scans considerable diminution lesions but VSAA did not improve until performed hematopoietic stem cell transplantation 5 months later.Aplastic associated variety autoimmune syndromes. The sequence events patient suggests that failure, AFOP, may part an syndrome. could be result immune dysregulation pediatric case favorable outcome transplantation.