Alyson Bundy, DO
Saint Vincent Indianapolis Hospital
Indianapolis, Indiana
Disclosure information not submitted.
Brandon Pearce, D.O.
Dr. Brandon Pearce
Ascension St. Vincent Hospital, United States
Disclosure information not submitted.
Vrushank Patel, MD
Dr. Vru Patel
Ascension St. Vincent Hospital, United States
Disclosure information not submitted.
Title: An Interesting Case of Pulmonary Lymphomatoid Granulomatosis
Case Report Body:
Introduction:
Pulmonary lymphomatoid granulomatosis (LYG) is an EBV-driven lymphoproliferative disorder that presents as bilateral pulmonary nodules more common in men aged 40-70. Although difficult to diagnose, early diagnosis leads to improvement in mortality. Here we present an interesting case of LYG with rapidly expanding granulomas.
Description:
75-year-old male with a remote history of renal transplant and bilateral lung masses found 2 months prior presented to the ED with two days of confusion. Outpatient workup of the pulmonary masses included PET, EBUS, and CT-guided biopsy. Based on biopsies, the leading diagnosis was non necrotizing granuloma sarcoid-like reaction from immunosuppression and he was placed on prednisone. Malignancy could not be ruled out as the biopsy also demonstrated rare atypical cells. On presentation, initial workup revealed hyponatremia, thought to be SIADH, and an enlarging right lung mass. Brain MRI revealed abnormal areas of hyperintensity and multiple lesions with vasogenic edema. He was started on prophylactic Dexamethasone and Keppra and underwent extensive infectious disease and autoimmune workup, all of which was unrevealing. He then underwent a stereotactic brain biopsy. While awaiting results, the patient became obtunded and required ICU-level care for worsening encephalopathy. Ultimately brain biopsy revealed diffuse large B cell lymphoma and was positive for Epstein-Barr virus. The patient decompensated further and care was withdrawn.
Discussion:
LYG is difficult to diagnose due to its rarity and nonspecific symptoms that are similar to other pulmonary processes. It often requires multiple biopsies and EBV RNA on biopsy samples to diagnose. Median survival is 14 months and the mortality rate is 60%. 15-25% of cases will evolve into aggressive lymphoma, which is almost always fatal. Early recognition and treatment is critical to achieve remission and avoid conversion to high-grade lymphoma. Providers must avoid anchoring biases and keep their differential diagnosis broad. Particularly when a patient does not respond appropriately to treatment, clinicians should consider alternative etiologies such as LYG. Maintaining a broad differential, especially when faced with treatment failure, can be life-saving as many disease processes such as LYG rely on prompt diagnosis.