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Autoimmunity and Delayed Diagnosis in Pediatric Idiopathic Pulmonary Hemosiderosis

AlJassmi, Abdulrahman M. MBBS, MD, FACHARZT, MBA

Journal of Pediatric Hematology/Oncology: May 15, 2019 - Volume Publish Ahead of Print - Issue - p
doi: 10.1097/MPH.0000000000001513
Clinical and Laboratory Observations: PDF Only

Idiopathic pulmonary hemosiderosis is characterized by a triad of iron-deficiency anemia, hemoptysis, and radiographic diffuse lung infiltrates. However, the inconsistent initial presentation in children may cause a significant delay in diagnosis. Autoimmune reactivity seems to be the most acceptable theory of pathogenesis. We reported an 8-year-old boy presenting with a cough, fever, and difficulty breathing with a history of iron-deficiency anemia and an abnormal autoimmune response in the last 3 years. Perinuclear antineutrophil cytoplasmic antibodies were positive and chest computed tomography revealed patchy ground glass haziness. Bronchoalveolar lavage fluid showed hemosiderin-laden macrophages. The respiratory symptoms improved with oral corticosteroids.

Pediatric Hematology/Oncology Department, Dubai Hospital, Dubai Health Authority, Dubai, United Arab Emirates

The author declares no conflict of interest.

Reprints: Abdulrahman M. Al Jassmi, MBBS, MD, FACHARZT, MBA, Pediatric Hematology/Oncology Department, Dubai Hospital, Dubai Health Authority, P.O. Box 92227 Dubai, United Arab Emirates (e-mail:

Received September 10, 2018

Accepted April 15, 2019

Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.