Pulmonary Alveolar Proteinosis in Association with Secondary Hemophagocytic Lymphohistiocytosis

Pulmonary alveolar proteinosis (PAP) is a rare diffuse lung disease in the pediatric population. There are currently few cases documenting hemophagocytic lymphohistiocytosis as a cause for secondary PAP. We describe an ex-preterm child with secondary hemophagocytic lymphohistiocytosis, complicated b...

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Bibliographic Details
Published in:	The Journal of pediatrics Vol. 183; pp. 191 - 195
Main Authors:	Lin, Jenny, MD, De, Aliva, MD, Figueiredo, Lisa, MD, Maxwell, Rochelle, MD, Wasserman, Emily, MD, Adams, Kelly, DO, Weingarten, Jacqueline, MD, Peek, Giles, MD, Miksa, Michael, MD
Format:	Journal Article
Language:	English
Published:	United States Elsevier Inc 01-04-2017
Subjects:	Abnormalities, Multiple - diagnosis Abnormalities, Multiple - therapy Biopsy, Needle Combined Modality Therapy Female Follow-Up Studies Humans Immunohistochemistry Infant Lymphohistiocytosis, Hemophagocytic - complications Lymphohistiocytosis, Hemophagocytic - diagnosis Lymphohistiocytosis, Hemophagocytic - therapy Pediatrics Pulmonary Alveolar Proteinosis - complications Pulmonary Alveolar Proteinosis - diagnosis Pulmonary Alveolar Proteinosis - therapy Radiography, Thoracic - methods Rare Diseases Respiration, Artificial Respiratory Insufficiency - diagnosis Respiratory Insufficiency - etiology Respiratory Insufficiency - therapy Risk Assessment Severity of Illness Index Tomography, X-Ray Computed - methods Tracheostomy - methods Treatment Outcome ECMO FUO Extracorporeal membrane oxygenation Granulocyte-macrophage colony-stimulating factor HLH Langerhans cell histiocytosis Fraction of inspired oxygen LCH Natural killer cell CT Hemophagocytic lymphohistiocytosis Computed tomography Pulmonary alveolar proteinosis FiO 2 Fever of unknown origin GM-CSF WLL Whole lung lavage EBV Epstein-Barr virus NK MAS PAP Macrophage activation syndrome FiO2
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Summary:	Pulmonary alveolar proteinosis (PAP) is a rare diffuse lung disease in the pediatric population. There are currently few cases documenting hemophagocytic lymphohistiocytosis as a cause for secondary PAP. We describe an ex-preterm child with secondary hemophagocytic lymphohistiocytosis, complicated by PAP and hypoxemic respiratory failure.
Bibliography:	ObjectType-Case Study-3 SourceType-Scholarly Journals-1 content type line 23 ObjectType-Review-1 ObjectType-Feature-5 ObjectType-Report-2 ObjectType-Article-4
ISSN:	0022-3476 1097-6833
DOI:	10.1016/j.jpeds.2016.12.037