Secondary pulmonary alveolar proteinosis associated with primary myelofibrosis and ruxolitinib treatment: An autopsy case

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Abstract

Pulmonary alveolar proteinosis (PAP) is an uncommon lung disorder characterized by the excessive accumulation of surfactant-derived lipoproteins in the pulmonary alveoli and terminal bronchiole. Secondary PAP associated with primary myelofibrosis (PMF) is extremely rare, and to our knowledge, no autopsy case has been reported. We herein report an autopsy case of secondary PAP occurring in a patient with PMF who was treated with the Janus kinase 1/2 inhibitor ruxolitinib. We confirmed a diagnosis of PAP with complications based on the pathological findings at the autopsy. Notably, this case might suggest an association between ruxolitinib treatment and PAP occurrence.

Original languageEnglish
Pages (from-to)2023-2028
Number of pages6
JournalInternal Medicine
Volume59
Issue number16
DOIs
Publication statusPublished - 2020

Keywords

  • Autopsy
  • Primary myelofibrosis
  • Pulmonary alveolar proteinosis
  • Ruxolitinib

ASJC Scopus subject areas

  • Internal Medicine

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