SESSION TITLE: Pulmonary Manifestations of Systemic Disease 1 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Lymphomatoid granulomatosis (LYG) is a rare Epstein-Barr virus (EBV)-driven lymphoproliferative disease. Clinical manifestations often mimic infectious diseases, malignancy and vasculitis, frequently leading to diagnostic delays. CASE PRESENTATION: A 53-year-old Chinese man with recurrent perianal abscesses, was found to have bilateral nodular opacities on a pre-operative chest radiograph (Figure 1A). Shortly after, he developed an acute onset of diplopia. On examination, bilateral cranial nerve 6, and left cranial nerve 7 and 8 palsies were noted. His medical history was notable for polymyositis treated with mycophenolate mofetil and prednisolone for the past 4 years. Imaging of the thorax was consistent with bilateral pulmonary nodules of varying sizes, and several demonstrated cavitation (Figure 1). Magnetic resonance imaging of the brain detected T2/FLAIR hyper intense signals in the left cerebellar superior sulci, left hemi pons and right 6th cranial nerve (Figure 1). Lumbar puncture was performed, and cerebrospinal fluid cytological examination yielded atypical CD20+ B cells. Transbronchial lung biopsy revealed florid fibrinohemorrhagic exudates and polymorphous infiltrates consisting of numerous B lymphocytes, some T lymphocytes, histiocytes and scattered atypical large lymphoid cells, focally involving vessel walls. EBV by in situ hybridization showed diffuse positive reaction (Figure 2). Histology of the recurring perianal abscess reported at another institution was reviewed at our hospital, demonstrating involvement by LYG. Fungal and acid fast bacilli stains were negative. A diagnosis of LYG, grade 3 was made. DISCUSSION: LYG is a rare EBV-associated lymphoproliferative disorder. The lungs are almost invariably affected, presenting classically as bilateral pulmonary nodules1. Central nervous system (CNS) involvement occurs in one-third of patients, and portends a poor prognosis1. Our patient presented with synchronous pulmonary, CNS and perianal skin/soft tissue involvement, which is uncommon in LYG. Reported associations of LYG are immune-related (autoimmune conditions, immunodeficiency states e.g. common variable immunodeficiency)1, and it is postulated that immune dysregulation may result in inadequate control of EBV infected B-cells. Our patient was immunosuppressed with mycophenolate mofetil, which may have contributed to the development of LYG. Management involves a consideration for stopping immunosuppression if possible, and chemotherapy which should be started immediately in high grade disease or CNS involvement. CONCLUSIONS: We describe a rare case of multi-systemic LYG with bilateral cavitary nodules, cranial nerve palsies, and recurring perianal abscess. Differential diagnoses for multiple pulmonary nodules are extensive. However, the possibility of LYG should be suspected, especially in immunosuppressed patients. Reference #1: Roschewski M, Wilson WH. Lymphomatoid granulomatosis. Cancer J. 2012; 18(5):469-74. DISCLOSURE: The following authors have nothing to disclose: Ken Junyang Goh, Angela Takano, Pei Yee Tiew, Ming Hui Yong, Chee Kiang Tay No Product/Research Disclosure Information