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

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

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 phagocyti...

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Main Authors: Forat Lutfi, Arpan Patel, Dawn Becker, Mohammad Shahid, Kairav Shah
Format: Article
Language:English
Published: Elsevier 2018-01-01
Series:IDCases
Online Access:http://www.sciencedirect.com/science/article/pii/S2214250918300222
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spelling doaj-1832754e3c68470e9b6e77f403491cfb2021-07-02T11:00:32ZengElsevierIDCases2214-25092018-01-0114Hemophagocytic lymphohistiocytosis (HLH) presenting as fever of unknown origin and acute liver failureForat Lutfi0Arpan Patel1Dawn Becker2Mohammad Shahid3Kairav Shah4University of Florida Department of Medicine, Gainesville, FL, United States; Corresponding author.University of Florida Department of Medicine, Division of Hematology/Oncology, Gainesville, FL, United StatesUniversity of Florida Department of Medicine, Division of Infectious Diseases & Global Medicine, Gainesville, FL, United StatesUniversity of Florida Department of Pathology, Gainesville, FL, United StatesUniversity of Florida Department of Medicine, Division of Infectious Diseases & Global Medicine, Gainesville, FL, United StatesA 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: HLH, Acute EBV infection, Acute liver failure, Cytokine stormhttp://www.sciencedirect.com/science/article/pii/S2214250918300222
collection DOAJ
language English
format Article
sources DOAJ
author Forat Lutfi
Arpan Patel
Dawn Becker
Mohammad Shahid
Kairav Shah
spellingShingle Forat Lutfi
Arpan Patel
Dawn Becker
Mohammad Shahid
Kairav Shah
Hemophagocytic lymphohistiocytosis (HLH) presenting as fever of unknown origin and acute liver failure
IDCases
author_facet Forat Lutfi
Arpan Patel
Dawn Becker
Mohammad Shahid
Kairav Shah
author_sort Forat Lutfi
title Hemophagocytic lymphohistiocytosis (HLH) presenting as fever of unknown origin and acute liver failure
title_short Hemophagocytic lymphohistiocytosis (HLH) presenting as fever of unknown origin and acute liver failure
title_full Hemophagocytic lymphohistiocytosis (HLH) presenting as fever of unknown origin and acute liver failure
title_fullStr Hemophagocytic lymphohistiocytosis (HLH) presenting as fever of unknown origin and acute liver failure
title_full_unstemmed Hemophagocytic lymphohistiocytosis (HLH) presenting as fever of unknown origin and acute liver failure
title_sort hemophagocytic lymphohistiocytosis (hlh) presenting as fever of unknown origin and acute liver failure
publisher Elsevier
series IDCases
issn 2214-2509
publishDate 2018-01-01
description 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: HLH, Acute EBV infection, Acute liver failure, Cytokine storm
url http://www.sciencedirect.com/science/article/pii/S2214250918300222
work_keys_str_mv AT foratlutfi hemophagocyticlymphohistiocytosishlhpresentingasfeverofunknownoriginandacuteliverfailure
AT arpanpatel hemophagocyticlymphohistiocytosishlhpresentingasfeverofunknownoriginandacuteliverfailure
AT dawnbecker hemophagocyticlymphohistiocytosishlhpresentingasfeverofunknownoriginandacuteliverfailure
AT mohammadshahid hemophagocyticlymphohistiocytosishlhpresentingasfeverofunknownoriginandacuteliverfailure
AT kairavshah hemophagocyticlymphohistiocytosishlhpresentingasfeverofunknownoriginandacuteliverfailure
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