SESSION TITLE: Medical Student/Resident Pulmonary Manifestations of Systemic Disease 2 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening syndrome which results from excessive immune activation. Infection is a usual trigger in patients with genetic predisposition, but sporadic cases have also been reported. Mostly seen in infants and children, it commonly presents with fever, hepatitis, encephalitis, rash and lymphadenopathy. Pulmonary involvement is uncommon but can present as interstitial infiltrates and pulmonary edema. We present a case of a young male with pulmonary embolism, found to have HLH from EBV. CASE PRESENTATION: 22 y/o male presented to the hospital with fever and dyspnea for two days. He had a viral infection about a month ago which had resolved. Labs showed leukopenia and thrombocytopenia as well as hepatitis with elevated transaminases. A CTA thorax found Pulmonary emboli in the right upper and lower lobes. He was started on enoxaparin for the PE. After two days he had hemoptysis from pulmonary hemorrhage and his pancytopenia worsened. Enoxaparin was stopped and a bone marrow biopsy was done which showed patchy reticulin fibrosis and increased histiocytes and megakaryocytes suggestive of hemophagocytic syndrome. He fit >5 of 8 criteria for HLH with Fever, cytopenias, hypertriglyceridemia, hemophagocytosis, elevated ferritin and Interleukin 2 soluble receptor alpha (IL-2Rα) level at 22,789.Treatment with HLH-94 protocol was started with dexamethasone and etoposide & Rituximab was added as EBV titers were >1,000,000. He had an IVC filter placed as he could not be anticoagulated. He responded well initially to therapy with clinical improvement and reduction in IL-2Rα. His treatment was marred with multiple admissions and interruptions from febrile neutropenia, pancytopenia and DIC requiring transfusions with platelets, PRBC and FFP. Unfortunately he succumbed to his disease and died 3 months after he was diagnosed, from sepsis and pulmonary hemorrhage. DISCUSSION: HLH is a syndrome of unchecked immune activation which if left untreated, has a survival of only a few months, due to neurological complications, ARDS, sepsis and multi-organ failure. The greatest barrier to successful outcome for individuals with HLH is a delay in diagnosis. This patient was unfortunate to have severe HLH which lead to multiple complications, one of them being a pulmonary embolism. Anticoagulation was not a simple answer in his situation as the PE was caused by the coagulopathy from HLH thus resulting in pulmonary hemorrhage when he was anticoagulated. CONCLUSIONS: This case highlights the importance of recognizing the clinical features of HLH which can lack specificity, for a timely diagnosis and early administration of effective therapy. This case also urges us to find the cause of a VTE in a otherwise healthy patient with unprovoked VTE before instituting anticoagulation as it could result in disastrous consequences like pulmonary hemorrhage in this patient. Reference #1: Larroche C. Hemophagocytic lymphohistiocytosis in adults: diagnosis and treatment. Joint Bone Spine 2012; 79:356. DISCLOSURES: No relevant relationships by Maria Fariduddin, source=Web Response No relevant relationships by Muhammad Naqvi, source=Admin input No relevant relationships by Wajihuddin Syed, source=Admin input No relevant relationships by Kanica Yashi, source=Admin input