EPSTEIN BARR VIRUS INFECTION CAUSING SECONDARY HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS AND MULTIPLE ORGAN FAILURE

Abstract

SESSION TITLE: Wednesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/23/2019 09:45 AM - 10:45 AM INTRODUCTION: Hemophagocytic Lymphohistiocytosis (HLH) is a hyperinflammatory immune disorder explained by an overactivation of monocytes, lymphocytes and macrophages resulting in overproduction of cytokines. Secondary HLH is caused by infection, malignant tumors and autoimmune disease. Amongst these, 70% of cases are triggered by Epstein-Barr virus (EBV). We present a case of EBV infection leading to HLH and multiple organ failure. CASE PRESENTATION: 64-year-old gentleman with a past medical history significant for ischemic cardiomyopathy status post biventricular implantable cardioverter defibrillator, chronic kidney disease, peripheral arterial disease status post right above knee amputation initially presented as a transfer from an outside facility with concerns for septic shock. He was afebrile, blood pressure of 94/35 mm mercury, heart rate of 73/ minute. He required mechanical ventilation and vasopressors soon thereafter. Patient was not improving despite receiving broad-spectrum antibiotics and antifungals. Upon further investigation, his serum was positive for EBV viremia with low viral load . His liver enzymes were markedly elevated, Ferritin was >4500, triglycerides 316, Natural killer cell activity was 5 and CD25 (soluble IL-2) 14,131. Bone marrow biopsy showed increased histiocytes containing fully formed cellular debris, fully formed peripheral blood and bone marrow precursor elements representing hemophagocytosis. All other infectious, viral, fungal and tick-borne work up was negative. He was given 10 mg/meter square of dexamethasone and one dose of etoposide. He was transferred to a tertiary center for further management. Unfortunately - his condition markedly declined, and treatment was stopped prematurely with home hospice measures. DISCUSSION: HLH is the extreme end of the spectrum with uncontrolled hyperinflammation leading to multiorgan failure. In EBV-HLH the virus infects CD8+T cells. After infection, the cells express EBV latent membrane protein (LMP-1) causing massive cytokine release, promote T lymphocyte proliferation and uncontrolled activation of macrophages. Diagnosis is based on HLH-2004 which includes 5 of 8 criteria consisting of fever, splenomegaly, bicytopenias, hypertriglyceridemia, elevated ferritin, increased sCD25 (soluble IL-2), decreased or absent NK cell activity and/or Hemophagocytosis in bone marrow, CSF or lymph nodes. EBV-HLH is a rapidly progressing disease with one-year mortality as high as 75%. Treatment consists of dexamethasone and etoposide based on HLH-94 and HLH-2004 protocols. Early initiation of treatment is crucial regarding a favorable prognosis. CONCLUSIONS: HLH is a hyperinflammatory reaction most often caused by infection. High clinical suspicion is necessary when patients present with multiorgan failure with otherwise unexplained symptoms. Initiation of treatment may improve survival rates although often rapid deterioration is seen. Reference #1: Wang Y, Wang Z, Zhang J, et al. Genetic features of late onset primary hemophagocytic lymphohistiocytosis in adolescence or adulthood. PLoS One. 2014; 9(9);e107386 Reference #2: Maakaroun NR, Moanna A, Jacob JT, et al. Viral Infections associated with haemophagocytic syndrome. Rev Med Virol. 2010;20(2):93-105 Reference #3: Janka GE. Familial and acquired haemophagocytic lymphohistiocytosis. Eur J Pediatr. 2007:166(2);95-109 DISCLOSURES: No relevant relationships by Cristian Dumitrescu, source=Web Response No relevant relationships by Hafiza Wajeeha Javaid, source=Web Response No relevant relationships by Kunal Mehta, source=Web Response No relevant relationships by Farrukh Munir, source=Web Response No relevant relationships by Vipul Singh, source=Web Response