EXTRAORDINARY EXTRA NODAL LYMPHOMA

Abstract

SESSION TITLE: Monday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: There are over 20 sub types of non-Hodgkin Lymphoma (NHL), marginal zone lymphoma being the 3rd most common. Mucosa-associated lymphoid tissue lymphoma is an extra nodal marginal zone lymphoma (EMZL). We present a case of indolent nodal marginal zone lymphoma progressing to pulmonary EMZL with diffuse tracheobronchial involvement. CASE PRESENTATION: An 86-year-old female was seen in her oncologist’s office for follow up of indolent abdominal nodal marginal zone B cell lymphoma which had been stable for the last 5 years. She reported dry cough, exertional dyspnea and intermittent wheezing for the last 2 weeks. Medical history was unremarkable otherwise. Vital signs and physical exam were within normal limits. Baseline labs including CBC, CMP and chest x-ray were unremarkable. The patient was evaluated with contrast enhanced CT scan of the chest, abdomen and pelvis which revealed endobronchial lesion in the right middle and lower lobes with post obstructive atelectasis of the right middle lobe, and mediastinal, bilateral hilar and axillary lymphadenopathy. These findings were new compared to the previous CT scans. An endoscopic bronchial ultrasound was done which revealed diffuse mucosal involvement of the tracheobronchial tree in the form of nodular mucosa, and narrowing of the bronchus intermedius. Biopsy of the abnormal mucosa and thoracic lymphadenopathy was done and was consistent with marginal zone B cell lymphoma. Our patient was recommended induction with RCVP (rituximab, cyclophosphamide, vincristine, and prednisone) for 1-2 cycles for followed by maintenance with rituximab, revlimid or both. DISCUSSION: Marginal zone lymphoma (MZL) is a group of indolent NHL B-cell lymphomas, which accounts for approximately 8% of all NHL cases. The average age at diagnosis is 60 years, and it is slightly more common in women than in men. The most common form is EMZL. Common sites for EMZL are stomach, small intestine, salivary gland, thyroid and lungs. When present, EMZL of the lung will present as single or multiple nodules or consolidations on imaging. Treatment depends on the extent of the disease. Options are radiation, surgical resection, immunotherapy (anti-CD 20 monoclonal antibody) alone or in combination with chemotherapy. CONCLUSIONS: EMZL shares clinical features with chronic infections and metastatic cancer. It should be high in the differential in patients with consolidation and thoracic lymphadenopathy. Diffuse involvement of the airways including the upper airways is extremely rare which was present in our case. Also, our patient did not have any underlying autoimmune or infectious etiology that is found in most of the cases of EMZL. Long term success with any therapy is limited requiring close observation. Reference #1: Borie R, Wislez M, Antoine M, Bergman CC, Thieblemont C, Cadranel J. Pulmonary mucosa-associated lymphoid tissue lymphoma revisited. Eur Respir J 47: 1244-1260, 2016. Reference #2: Kawaguchi T, Himeji D, Kawano N, Shimao Y, Marutsuka K. Internal Medicine. 2018 Mar 9; 57(15): 2233-2236. Reference #3: Zinzani PL. The many faces of marginal zone lymphoma. Hematology Am Soc Hematol Educ Program1: 426-432, 2012. DISCLOSURES: No relevant relationships by Shujaa Faryad, source=Web Response No relevant relationships by Rajamurugan Meenakshisundaram, source=Web Response