2372 Atypical Infectious Mononucleosis From EBV in a Splenectomized Sickle Cell Disease Patient

Abstract

INTRODUCTION: Infectious mononucleosis (IM) is a clinical entity usually presenting with fever, pharyngitis and lymphadenopathy. However, atypical features of IM in Sickle cell disease (SCD) patients post splenectomy have been rarely reported. Here, we present a case of an atypical IM from Epstein Barr Virus (EBV) in a SCD patient post splenectomy with concurrent sickle cell hepatopathy (SCH). CASE DESCRIPTION/METHODS: This is a 22-year-old young man with history of SCD post splenectomy, who presented with severe dull pain in his right knee and persistent fevers up to 38.3oC along with watery diarrhea for 2-3 days. The patient denied any sore throat, sick contacts or recent blood transfusions. Besides a history of transient hemochromatosis from blood transfusions, the patient denied any history of liver disease or immunosuppression. Clinical examination was significant for non-tender hepatomegaly and a small palpable lymph node in the left posterior cervical chain. Initial workup was significant for lymphocytosis, mild transaminitis with raised AST, ALT, ALP, total bilirubin and direct bilirubin. Blood and urine cultures, parasitic screen, hepatitis and autoimmune hepatic panel were all unremarkable. Imaging studies were only significant for hepatomegaly on Abdominal Ultrasound. Despite initial management with broad spectrum antibiotics, the patient continued to have high fevers and rising liver transaminitis. Subsequent investigations revealed a positive EBV IgM and a viral load of 6119 along with 19% atypical lymphocytes. The patient was diagnosed with atypical EBV infection with mild SCH and managed supportively with IV fluids, hydroxyurea and analgesics. DISCUSSION: IM often presents as a clinical triad of fever, pharyngitis and lymphadenopathy with EBV being the most common pathogen implicated. EBV has often been associated with a mild transaminitis in healthy adults. A similar pattern is also seen in SCD patients with SCH resulting in tender hepatomegaly secondary to vaso-occlusive hepatic ischemia. Hence, it can be difficult to distinguish an atypical presentation of EBV-IM from SCH. In our patient, although his liver transaminitis partially resolved post exchange transfusion, he continued to have rising fever and atypical lymphocytosis along with high EBV viral load indicating an active atypical EBV-IM with a concurrent mild SCH. Therefore, any mild transaminitis with cholestasis in SCD patients post splenectomy, should prompt an evaluation for atypical EBV-IM.