ANGIOIMMUNOBLASTIC T-CELL LYMPHOMA AS A CAUSE OF PERSISTENT PULMONARY HYPERTENSION

Abstract

SESSION TITLE: Medical Student/Resident Pulmonary Vascular Disease 1 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Angioimmunoblastic T-cell lymphoma (AITL) is rare, accounting for 15-20% of peripheral T-cell lymphomas. Diagnosis can be challenging, as AITL may present with symptoms such as rash and arthralgias more suggestive of an autoimmune disorder (1). There are very few published reports of lymphoma-associated pulmonary hypertension (PH), most involving intravascular B-cell lymphomas (2). Here we present a case of AITL-associated PH that did not resolve with lymphoma remission. CASE PRESENTATION: A previously healthy 63-year-old woman presented after weeks of fevers, arthralgias, rashes, and dyspnea on exertion. Pulmonary function testing and CT chest were initially unremarkable. After an equivocal work-up she was provisionally diagnosed with Still’s disease, but saw no improvement on colchicine or anakinra. FDG-PET obtained a year after presentation revealed new pleural and pericardial effusions, splenomegaly, and diffuse lymphadenopathy, but no abnormal uptake in the lungs. Echocardiogram revealed mild RV enlargement with reduced function and an estimated RVSP of 73. A lymph node biopsy was consistent with AITL, a diagnosis further supported by the presence of a TCR gene rearrangement. She was started on chemotherapy with CEOP and had complete response after 2 cycles. Despite her excellent response to chemotherapy, her dyspnea rapidly progressed and repeat PFTs showed interval development of a moderately reduced diffusion capacity. Repeat CT chest was again negative for pulmonary infiltrates and V/Q scan did not suggest chronic embolism. On physical exam she now had JVD, 2+ pitting edema to her knees, and cool extremities. Right heart catheterization confirmed severe PH with mPAP 46 mmHg, normal PAWP, and PVR 13 WU. A thorough evaluation for other causes of PH was unrevealing. The patient’s symptoms improved on PDE5i and ERA therapy; HRCT did not reveal signs of PVOD nor did she develop hypoxia on treatment. DISCUSSION: Lymphoma is a rare cause of PH, and to our knowledge this is only the second reported case of PH due to AITL (3). In that case, as well as in other cases of lymphoma-associated PH, the PH improved or resolved with lymphoma remission. Our patient’s PH continued even after complete response to chemotherapy, and we hypothesize that the delay in diagnosis allowed time for fixed remodeling of the pulmonary arterial endothelium. CONCLUSIONS: Lymphoma may be an underappreciated cause of PH, and should be considered in patients with unexplained PH. AITL, in particular, should be considered in patients with PAH and symptoms suggestive of a systemic rheumatologic disorder. Imaging devoid of interstitial changes does not preclude this diagnosis, particularly once PE, PVOD, and pulmonary capillary hemangiomatosis have been considered. Treatment with PAH therapy was safe and effective in this case. Reference #1: de Leval et al. Br J Haematol. 2010 Mar; 148(5): 673-89 Reference #2: Nelson et al. J Thorac Oncol. 2012 Dec; 7(12): e34-5 Reference #3: Hua J et al. J Hematol Malig. 2012; 2(3): 26. DISCLOSURES: No relevant relationships by Anna Bracewell, source=Web Response No relevant relationships by Sachin Gupta, source=Web Response