HOW HIGH CAN FERRITIN LEVEL BE? A RARE CASE OF EHRLICHIOSIS-INDUCED HLH WITH REACTIVATION OF EBV

Abstract

SESSION TITLE: Critical Care 3 SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Hemophagocytic lymphohistocytosis (HLH) is often under recognized which leads to high mortality and morbidity. Prompt recognition and treatment can decrease mortality. We present a case of HLH triggered by infection with Ehrlichia with reactivation of EBV. CASE PRESENTATION: A 28-year-old man with no past medical history presented with fever, chills, generalized musculoskeletal pains, nausea, vomiting, loose stools and headache. He went to hiking at Roanoke river and Greenway a week prior to presentation. Physical exam revealed blood pressure 123/69 mmHg, pulse 96/min, temperature 103 F, respiratory rate 36/min. Pertinent physical examination findings include cervical lymphadenopathy, generalized muscle tenderness with bilateral shin petechiae. Laboratory data revealed leukopenia 0.9, platelets 17, transaminitis (AST-457, ALT- 96), CK- 8663, fibrinogen 162, and ferritin 61,437. He was admitted for neutropenic fever and was empirically started on vancomycin, cefepime and doxycycline for tick borne infection. Bone marrow biopsy was performed due to concern for HLH revealed histocytes with intracellular RBC and WBC’s. Further workup is negative for any autoimmune disease, malignancies, but positive for IgG EBV and Ehrlichia. PCR for EBV DNA was positive with viral load 2096 copies/ml (normal < 200 copies/ml). Received a dose of Etoposide and Decadron but he started to improve clinically. Doxycycline was continued and rest of the antibiotics and chemotherapy was discontinued. Patient was discharge in stable condition and completed total 14 days of doxycycline treatment. Patient remained asymptomatic one month after hospital discharge. DISCUSSION: HLH is a life-threatening disorder characterized by unregulated hyperimmune response to antigen which results in phagocytosis of white cells, RBCs and platelets by mononuclear macrophages. It was first described in 1952 by Farquhar and Claireaux. Secondary causes of HLH is seen in adults from autoimmune diseases, infections and malignancies. Viral infections are the most common triggers: EBV, CMV, HIV. Elevated ferritin >10,000 μg/L has been demonstrated to be 90% sensitive and 96% specific for HLH (3). If left untreated mortality is high with median survival less than 2 months. HLH-1994 treatment protocol recommended an 8-week induction with IV dexamethasone and etoposide, and HLH-2004 protocol added cyclosporine to help prevent relapse.Most patients respond to early initiation of doxycycline 100 mg twice daily in Ehrlichiosis induced HLH and can be continued 4-5 days after clinically resolution of symptoms. If refractory then can use chemotherapeutic agents like etoposide, cyclosporine, intrathecal methotrexate, methylprednisolone. CONCLUSIONS: HLH should be in differential diagnosis in patients who present from endemic regions of tick borne infections with fever, cytopenia’s and especially with high ferritin levels (> 500 μg/L). Reference #1: Ehrlichia-Induced Hemophagocytic Lymphohistiocytosis: A Case Series. Zaher K et al. Blood 2014 124:4105 Reference #2: Ehrlichiosis-Induced Hemophagocytic Lymphohistiocytosis Presenting As Septic Shock - An Immune Response Gone Haywire And Its Lessons For The Icu Setting. N. G. Holtzman et al. Am J Respir Crit Care Med 189;2014: A6737 Reference #3: Hemophagocytic lymphohistiocytosis: review of etiologies and management. Melissa R George. J Blood Med. 2014; 5: 69–86. Published online 2014 Jun 12. https://doi.org/10.2147/JBM.S46255 DISCLOSURES: No relevant relationships by Venkateswara Kollipara, source=Web Response No relevant relationships by Umar Sofi, source=Web Response