CHEST Guidelines-A-23-Year-Old-Man-With-Multilobar-Consolidation

A-23-Year-Old-Man-With-Multilobar-Consolidation_ch

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A 23-year-old man was repeatedly admitted to the emergency department with symptoms of respiratory distress, cough, and fever, initially treated for pulmonary edema attributed to mitral regurgitation with mitral valve prolapse. Despite treatment with antibiotics and diuretics, the symptoms recurred persistently over several months. Upon detailed evaluation during his latest admission, significant eosinophilia was noted with an elevated white blood cell count and the presence of the FIP1L1-PDGFRA fusion mutation, indicative of primary myeloproliferative hypereosinophilic syndrome (HES).<br /><br />His condition was marked by consolidated lung opacities with ground-glass appearances and mediastinal lymph node enhancement. A bronchoalveolar lavage and transbronchial lung biopsy confirmed eosinophilic inflammation. Spirometry suggested restrictive lung disease, which, along with the reduced diffusing capacity of the lung for carbon monoxide, indicated parenchymal pathology.<br /><br />HES is a systemic condition predominantly affecting individuals aged between 20 and 40, more common in men, characterized by unexplained persistent eosinophilia leading to tissue and multiorgan damage. Pulmonary involvement is a common manifestation, highlighted by symptoms like chronic cough and dyspnea. Differential diagnoses include conditions with similar eosinophilic presentations such as eosinophilic granulomatosis with polyangiitis and chronic eosinophilic pneumonia.<br /><br />Upon confirming HES with the FIP1L1-PDGFRA mutation, the patient was treated with imatinib mesylate, a tyrosine kinase inhibitor. His symptoms notably improved, and a follow-up CT scan showed complete resolution of pulmonary infiltrates.<br /><br />This case emphasizes the importance of considering HES in persistent eosinophilia cases, particularly those not responding to conventional treatments, and highlights effective management with targeted therapies like imatinib for cases with specific genetic mutations.

Keywords

hypereosinophilic syndrome

FIP1L1-PDGFRA mutation

eosinophilia

imatinib mesylate

pulmonary involvement

mitral valve prolapse

tyrosine kinase inhibitor

respiratory distress

restrictive lung disease

eosinophilic inflammation