Pulmonary Alveolar Proteinosis in Association with Secondary Hemophagocytic Lymphohistiocytosis

J Pediatr. 2017 Apr:183:191-195. doi: 10.1016/j.jpeds.2016.12.037. Epub 2017 Jan 12.

Abstract

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.

Publication types

  • Case Reports
  • Review

MeSH terms

  • 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*
  • 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