Pulmonary lymphomatoid granulomatosis: An uncommon disease but not to be forgotten—a single centre experience

Abstract

Pulmonary lymphomatoid granulomatosis (PLG) is a rare multisystem Epstein–Barr virus (EBV)‐associated lymphoproliferative disorder. Exact incidence is unknown and, with its variable clinical presentation, making an accurate diagnosis of PLG can be difficult. We present two distinct cases at our tertiary centre that underline PLG's non‐specific clinical presentations. This resulted in the failure of recognizing PLG early with consequently progressive fatal outcomes. The rationale is to enlighten us concisely the knowledge surrounding PLG and consider it as a potential differential diagnosis, particularly in those immunosuppressed patients with radiological evidence of worsening pulmonary infiltrates not responding to customary treatment for common diagnoses. Having a high degree of suspicion for PLG in the right setting and pursuing lung biopsy early if appropriate for histopathology examination would be justified. This is essential to correctly diagnose PLG up‐front and subsequently utilize best management approach for a better survival and mortality risk outlook.

Acute respiratory failure developing in a patient with lymphomatoid granulomatosis

“…Only a few cases of LYG with acute progressive failure have been reported 3 8 9 13 14. A biopsy could not be performed in most patients, resulting in a fatal clinical course without a definitive diagnosis.…”