A tricky and rare cause of pulmonary eosinophilia: Myeloid/lymphoid neoplasm with eosinophilia and rearrangement of PDGFRA

Magda Zanelli, Maxwell Smith, Maurizio Zizzo, Angelo Carloni, Riccardo Valli, Loredana De Marco, Moira Foroni, Andrea Palicelli, Giovanni Martino, Stefano Ascani

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Eosinophilic lung diseases represent a heterogeneous group of disorders with prominent infiltrate of eosinophils in lung interstitium and alveolar spaces. Peripheral blood eosinophilia is often present. Infections, drugs, allergens, toxic agents have to be evaluated as possible causes of eosinophilic lung infiltrates. The category of myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRA, PDGFRB, FGFR1 and PCM1-JAK2 represents an uncommon cause of eosinophilic lung infiltrate. Case presentation: We report the case of a 70-year old man complaining of dry cough and dyspnea. Ground glass-opacities were seen on imaging studies and peripheral blood eosinophilia was present. A thorough step-wise patient's evaluation led to identify the clonal nature of eosinophilia and the diagnosis of myeloid/lymphoid neoplasm with eosinophilia and rearrangement of PDGFRA was made. Conclusions: Correlation with clinical history, laboratory tests and imaging studies is essential to achieve the correct diagnosis when facing with eosinophilic lung infiltrates. A prolonged eosinophilia can cause life-threatening organ damage. Identification of PDGFRA rearrangement, as in the present case, is particularly critical given the sensitivity and excellent response to imatinib, which has completely changed the natural history of this neoplasm.

Original languageEnglish
Article number216
JournalBMC Pulmonary Medicine
Volume19
Issue number1
DOIs
Publication statusPublished - Nov 19 2019

Keywords

  • Eosinophilia
  • Lung
  • Lymphoid
  • Myeloid
  • Neoplasm
  • PDGFRA

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

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