Primary Pulmonary Anaplastic Large Cell Lymphoma In A Young Male

SESSION TITLE: Fellows Lung Cancer Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Anaplastic large cell lymphoma (ALCL) is a type of non-Hodgkin lymphoma which is subclassified into primary systemic anaplastic lymphoma kinase (ALK)-positive, primary systemic ALK-negative, primary cutaneous ALCL, and breast implant-associated ALCL (1). ALCL is considered an extremely rare type of primary pulmonary lymphomas (2). We report a case of primary pulmonary ALCL in a young immunocompetent male presenting with dyspnea. CASE PRESENTATION: 43 year-old male with no past medical history presented to the emergency department with dyspnea, fatigue and lower extremity swelling which have been worsening over a month. Physical exam showed 3+ pitting edema bilaterally and bilateral decreased breath sound. Laboratory workup showed an elevate d-dimer and an elevated absolute monocytes. Chest X-ray showed numerous nodules throughout the lungs. CT angiogram of the chest, abdomen and pelvis showed a large infiltrative opacity extending from the right hilum to the pleural surface in the right lower lobe, with some extension into the central right middle lobe and innumerable pulmonary and hepatic metastases and lymphadenopathy in the thoracic, abdominal and pelvic lymph node chains. Tumor markers were negative, with mildly elevated CA-125. A right supraclavicular lymph node biopsy showed necrotizing lymphadenitis with no evidence of metastatic disease. Bronchoscopy revealed an extensive obstruction of the bronchial lumen with an endobronchial lesion in the right lower lobe bronchus. Tissue biopsy was consistent with anaplastic large cell lymphoma. Patient was critically ill in the intensive care unit and was intubated due to acute respiratory failure. He had a poor prognosis due to the extensive metastasis of his ALCL. DISCUSSION: ALCL is characterized by large lymphoid cells with pleomorphic nuclei, and uniform CD30/Ki-1 expression (2). The most common form of ALCL presents as primary cutaneous lymphoma and may secondarily involve the lungs. However, primary pulmonary ALCL is extremely rare with 10 published cases between 1990 and 2015 (3). ALK protein expression is an important prognostic indicator for ALCL as ALK-positive lymphomas have a favorable prognosis with a much higher 5-year survival rate (2). The diagnosis of ALCL includes histopathology and immunohistochemistry staining, with strong immunoreactivity for CD30. Our patient had a primary pulmonary ALCL which was ALK-negative, indicating poor overall prognosis, with a strong immunoreactivity for CD30 and Ki-1. The treatment of primary pulmonary ALCL has not been standardized. However, first-line therapy includes anthracycline-based regimen, which is the standard treatment for non-Hodgkin lymphomas. CONCLUSIONS: Our case underlines the importance of maintaining a high index of awareness for the occurrence and impact of the recognition of primary pulmonary ALCL and its implications on the clinical course of critically ill patients. Reference #1: Laurent, Camille; Haioun, Corinne; Brousset, Pierre; Gaulard, Philippe (1 September 2018). “New insights into breast implant-associated anaplastic large cell lymphoma”. Current Opinion in Oncology. 30 (5): 292–300. Reference #2: J.-F. Cordier, E. Chailleux, D. Lauque et al., “Primary pulmonary lymphomas; a clinical study of 70 cases in nonimmunocompromised patients,” CHEST, vol. 103, no. 1, pp. 201–208, 1993. Reference #3: Q. Zhao, Y. Liu, H. Chen et al., “Successful chemo-radiotherapy for primary anaplastic large cell lymphoma of the lung: A case report and literature review,” American Journal of Case Reports, vol. 17, pp. 70–75, 2016. DISCLOSURES: No relevant relationships by Obed Adarkwah, source=Web Response No relevant relationships by Raheel Anwar, source=Web Response No relevant relationships by Louis Gerolemou, source=Web Response No relevant relationships by Wael Kalaji, source=Web Response No relevant relationships by jad sargi, source=Web Response No relevant relationships by Viswanath Vasudevan, source=Web Response