Hemophagocytic lymphohistiocytosis (HLH) presenting as fever of unknown origin and acute liver failure

Forat Lutfi; Arpan Patel; Dawn Becker; Mohammad Shahid; Kairav Shah

doi:10.1016/j.idcr.2018.e00413

Hemophagocytic lymphohistiocytosis (HLH) presenting as fever of unknown origin and acute liver failure

IDCases. 2018 Jun 28:14:e00413. doi: 10.1016/j.idcr.2018.e00413. eCollection 2018.

Authors

Forat Lutfi¹, Arpan Patel², Dawn Becker³, Mohammad Shahid⁴, Kairav Shah³

Affiliations

¹ University of Florida Department of Medicine, Gainesville, FL, United States.
² University of Florida Department of Medicine, Division of Hematology/Oncology, Gainesville, FL, United States.
³ University of Florida Department of Medicine, Division of Infectious Diseases & Global Medicine, Gainesville, FL, United States.
⁴ University of Florida Department of Pathology, Gainesville, FL, United States.

Abstract

A 51-year-old previously healthy woman presenting with two-weeks of fever, flu-like symptoms, jaundice, and abdominal pain was found to have pancytopenia, transaminitis, and significantly elevated ferritin in the setting of an Epstein-Barr Virus (EBV) infection. Bone marrow biopsy revealed phagocytic macrophages consistent with findings of hemophagocytic lymphohistiocytosis (HLH). Given bone marrow findings and that the patient had five of the eight clinical criteria supporting the diagnosis of HLH, chemotherapy was initiated as per the HLH-94 protocol with initial improvement in patient's symptoms and overall functional status. This case demonstrates a classic presentation of HLH and displays the importance of correct diagnosis and prompt treatment.

Keywords: Acute EBV infection; Acute liver failure; Cytokine storm; HLH.

Publication types

Case Reports