Epstein-Barr virus induced haemophagocytic lymphohistiocytosis

Haemophagocytic lymphohistiocytosis (HLH) is a rare condition of uncontrolled immune activation as a result of an inherited genetic defect or in response to malignancy, autoimmune disease, rheumatological disease, AIDS infection or post-transplant immunosuppression. Described here is the case of a 1...

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Bibliographic Details
Published in:BMJ case reports 2021-05, Vol.14 (5), p.e241348
Main Authors: Zimmer, Markie, Gill, Inayat, Anusim, Nwabundo, Gaikazian, Susanna S
Format: Article
Language:English
Subjects:
Adult
Biopsy
Blood transfusions
Bone Marrow
Case Report
Case reports
Chemotherapy
Cytokine storm
Cytotoxicity
Disease
Epstein-Barr virus
Epstein-Barr Virus Infections - complications
Epstein-Barr Virus Infections - diagnosis
Epstein-Barr Virus Infections - drug therapy
Fever
Hemoglobin
Herpesvirus 4, Human
Humans
Immunology
Immunotherapy
Infections
Lymphohistiocytosis, Hemophagocytic - diagnosis
Lymphohistiocytosis, Hemophagocytic - drug therapy
Lymphohistiocytosis, Hemophagocytic - etiology
Male
Metabolism
Monoclonal antibodies
Patients
Pediatrics
Primary care
Serology
Young Adult
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Summary:Haemophagocytic lymphohistiocytosis (HLH) is a rare condition of uncontrolled immune activation as a result of an inherited genetic defect or in response to malignancy, autoimmune disease, rheumatological disease, AIDS infection or post-transplant immunosuppression. Described here is the case of a 19-year-old Caucasian man who presented with complaints of worsening fever, new-onset jaundice and lethargy after failing treatment for suspected infectious mononucleosis. Physical examination was significant for fever and splenomegaly while laboratory results revealed transaminitis, cytopaenia, indirect hyperbilirubinaemia and elevated ferritin, raising the likelihood of both autoimmune haemolytic anaemia and HLH. He tested positive for Epstein-Barr virus (EBV), and bone marrow biopsy revealed hypercellular marrow with haemophagocytosis and no evidence of malignancy. High dose steroids were initiated with significant improvement in haemoglobin, resulting in a final diagnosis of HLH secondary to acute EBV infection. The patient was discharged on continued high-dose prednisone with planned taper and consideration of outpatient rituximab therapy for 4 weeks. High clinical suspicion and prompt evaluation were critical to early treatment and decreased morbidity.
ISSN:1757-790X
1757-790X
DOI:10.1136/bcr-2020-241348