CARDIAC TAMPONADE AS A PRESENTING FEATURE OF METASTATIC GI CARCINOMA

Abstract

TOPIC: Critical Care TYPE: Fellow Case Reports INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening disease. It is characterized by abnormalities in immunoregulation due to a non-malignant proliferation of lymphocytes and secretion of inflammatory cytokines. HLH is typically associated with acquired immunodeficiencies such as in hematopoietic stem cell transplantation. We present a case of a young woman who was treated in our ICU for confirmed HLH which was attributed to an Epstein-Barr virus (EBV) infection that reactivated during her post-transplant immunosuppression. CASE PRESENTATION: A 46-year-old female presented to her primary care physician with gradual dyspnea, spontaneous gum bleeding and lower extremity bruising. She was diagnosed with anemia during her pregnancy. Eventually developed leukopenia and pancytopenia. She was later diagnosed with aplastic anemia and underwent a conditioning regimen prior to peripheral blood stem cell transplantation (PBSCT) with cyclophosphamide, fludarabine, total body irradiation, and rabbit anti-thymocyte globulin (ATG). She had an allogeneic PBSCT from a 10/10 HLA-matched unrelated donor (MUD). Her pre-transplant workup was positive EBV IgG. Donor pre-transplant workup was negative for EBV. Post-transplant immunosuppression consisted of rituximab and tacrolimus. Two months post-transplant, she was admitted for non-neutropenic fevers and diagnosed with Clostridium difficile colitis, acute kidney injury, EBV viremia (>800000 IU/ml), and widespread lymphadenopathy concerning for EBV-associated biopsy proven monomorphic PTLD. She was treated with two doses of Rituximab. Due to worsening encephalopathy, liver function tests and metabolic acidosis, ICU transfer initiated for continuous renal replacement therapy (CRRT). She developed acute respiratory failure and septic shock; was intubated, requiring vasopressors. During her ICU stay, HLH was considered. Bone marrow aspiration and biopsy performed confirmed HLH. High dose dexamethasone therapy was initiated. She also received two doses of Etoposide and a dose of tocilizumab in ICU. She was successfully extubated to high flow nasal cannula. Next Day, given her worsening mental status; Emapalumab (GAMIFANT, Novimmune SA), a fully human anti–IFN-gamma monoclonal antibody for HLH, was administered. Her cognitive function further declined, and she progressed to multiorgan failure and death. DISCUSSION: Aplastic anemia is a hematologic disorder characterized by pancytopenia and bone marrow aplasia. Bone marrow transplantation and immunosuppressive therapy are the modalities currently used, with success rates ranging 60% to 80%. Most treatment options for EBV-HLH have unclear effectiveness in adults. CONCLUSIONS: Despite trying rituximab, dexamethasone, etoposide, tocilizumab and Emapalumab, our transplant patient exhibits a unique and ambiguous intersection of EBV-associated refractory HLH, unresponsive to treatments. REFERENCE #1: Imashuku S, Kuriyama K, Sakai R, Nakao Y, Masuda S, Yasuda N, et al. Treatment of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) in young adults: a report from the HLH study center. Med Pediatr Oncol. 2003;41(2):103-9. REFERENCE #2: Fox CP, Shannon-Lowe C, Gothard P, Kishore B, Neilson J, O'Connor N, et al. Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in adults characterized by high viral genome load within circulating natural killer cells. Clin Infect Dis. 2010;51(1):66-9. REFERENCE #3: Locatelli F, Jordan MB, Allen C, Cesaro S, Rizzari C, Rao A, et al. Emapalumab in Children with Primary Hemophagocytic Lymphohistiocytosis. N Engl J Med. 2020;382(19):1811-22. DISCLOSURES: No relevant relationships by Padmastuti Akella, source=Web Response No relevant relationships by Saira Imran, source=Web Response No relevant relationships by Aizaaz Khan, source=Web Response No relevant relationships by Kevin Lazo, source=Web Response