RARE COMPLICATION OF A RARE DISEASE

Abstract

SESSION TITLE: Pulmonary Pathology II SESSION TYPE: Med Student/Res Case Report PRESENTED ON: 10/21/2019 3:15 PM - 4:15 PM INTRODUCTION: Primary pulmonary lymphoma (PPL) comprises of only 1% of pulmonary malignancies and has been described with systemic autoimmune diseases. It may be associated with a fatal disease known as intravascular lymphoma which is extremely rare and can lead to a diagnostic dilemma. CASE PRESENTATION: 58 year old female with history of Sjögren’s syndrome presented to the pulmonology clinic with complaints of shortness of breath cough and low grade fever for 2 weeks. She was vitally stable with bronchial breath sounds at the right lower lung zone. Chest X-ray showed right middle lobe collapse. The CT scan chest after 2 weeks of conservative treatment showed soft tissue density in the bronchus intermedius (BI). Bronchoscopy revealed pinkish lesion in BI. The histopathology showed high grade B cell Non-Hodgkin's lymphoma. The patient was started on chemotherapy with R-CHOP (rituximab, cyclophosphamide, doxorubicin and prednisolone). After third cycle of chemotherapy she was admitted with fever and bodyaches. She became increasingly somnolent and dropped her GCS requiring intubation. The MRI brain showed diffusely scattered foci of signal dropout on SWI throughout the brain, predominantly in the corpus callosum and diffuse multifocal areas of diffusion restriction in periventricular and subcortical white matter. Lumbar puncture and EEG were negative. Brain biopsy was not attempted considering her clinical instability. She was treated with pulse steroids leading to improvement in GCS. A follow up MRI after pulse therapy showed interval improvement of the brain lesions. DISCUSSION: We believe that our case describes the presentation of a malignancy which progressed to develop a rare complication known as Intravascular lymphoma (IVL); it is a subtype of extranodal large B-cell lymphoma which spreads in angiotropic pattern to virtually any organ of the body, brain and skin being the most common sites. The gold standard of diagnosis is histological evidence of intravascular invasion but studies are being reported to identify noninvasive modalities of diagnosis of IVL (1). Recent literature reports various neuroimaging findings of IVL like multiple foci of high signal intensity in the deep white matter, infarct like changes and intracranial mass (2). Treatment of IVL is based on the RCHOP regimen, however prognosis is poor (3). The responsiveness to steroid therapy supports the diagnosis of IVL. CONCLUSIONS: A high index of suspicion is required to diagnose IVL in patients with Non-Hodgkins who present with neurological symptoms. An emerging evidence supports the use of neuroimaging in diagnosis of IVL in patients without tissue diagnosis. Reference #1: Shimada K, Kinoshita T, Naoe T, Nakamura S. Presentation and management of intravascular large B-cell lymphoma. The lancet oncology. 2009 Sep 1;10(9):895-902 Reference #2: Yamamoto A, Kikuchi Y, Homma K, O'uchi T, Furui S. Characteristics of intravascular large B-cell lymphoma on cerebral MR imaging. American Journal of Neuroradiology. 2012 Feb 1;33(2):292-6 Reference #3: Baehring JM, Longtine J, Hochberg FH. A new approach to the diagnosis and treatment of intravascular lymphoma. Journal of neuro-oncology. 2003 Feb 1;61(3):237-48 DISCLOSURES: no disclosure on file for Arsalan Ahmad; No relevant relationships by Hasaan Omar, source=Web Response No relevant relationships by Aqusa Zahid, source=Web Response No relevant relationships by Ali Zubairi, source=Web Response