Haemophagocytic lymphohistiocytosis that spontaneously resolved: a case of EBV

• Published in: BMJ case reports 2020-11, Vol.13 (11), p.e235544
• Main Authors: Chahine, Zena, Jayakrishnan, Thejus, Samhouri, Yazan, Fazal, Salman
• Format: Article
• Language: English
• Subjects: Anti-Bacterial Agents - adverse effects; Anti-Bacterial Agents - therapeutic use; Antibiotics; Antigens, CD - immunology; Antigens, Differentiation, Myelomonocytic - immunology; Biopsy; Blood tests; Bone marrow; Bone Marrow - pathology; Case Report; Case reports; Chemotherapy; contraindications and precautions; Cytokines; Cytotoxicity; Diagnosis, Differential; Epstein-Barr Virus Infections - diagnosis; Female; Fever; haematology (incl blood transfusion); Hematology; Herpesvirus 4, Human - immunology; Humans; Immunoglobulin G - immunology; Immunoglobulin M - immunology; Infections; Lifesaving; Lymphocytes; Lymphohistiocytosis, Hemophagocytic - diagnosis; Lymphohistiocytosis, Hemophagocytic - drug therapy; Lymphohistiocytosis, Hemophagocytic - metabolism; pathology; Patients; Receptors, Interleukin-2 - metabolism; Remission, Spontaneous; Stem cell transplantation; Steroids; Transplants & implants; Withholding Treatment; Young Adult
• ISSN: 1757-790X; 1757-790X
• DOI: 10.1136/bcr-2020-235544

A 23-year-old Caucasian woman, presented with recurrent fevers, elevated liver function tests and pancytopenia. Her labs at presentation were white blood cells 1.5 ×109/L, haemoglobin 8 g/L, platelets 59 k/mcl, lactate dehydrogenase (LDH) over 2000 U/L, aspartate aminotransferase 593 U/L, alanine aminotransferase 1321 U/L, alkaline phosphatase 223 U/L and ferritin 7665 µg/L. Epstein-Barr virus (EBV) IgM and IgG antibodies were positive in serum. A soluble interleukin 2 receptor was elevated at 2458. A bone marrow biopsy revealed scattered macrophages containing erythrocytes and other cellular elements. Immunohistochemistry for CD68 highlighted macrophages with erythrophagocytosis and in situ hybridisation was positive for EBV. She met the diagnostic criteria for haemophagocytic lymphohistiocytosis (HLH). She was initially treated with broad spectrum antibiotics which were eventually discontinued once the diagnosis was established. Over a period of 2–3 weeks her fever, transaminitis, ferritin and LDH improved spontaneously. She continued to improve clinically and was subsequently discharged. HLH is an aggressive, life-threatening hyper-inflammatory syndrome which, if not promptly recognised and treated, can be fatal. Treatment involves etoposide-based chemotherapy and possible stem-cell transplantation. This patient showed signs of improvement spontaneously and a decision was made to not treat her. This was a rare case of EBV-associated HLH which resolved spontaneously without any intervention. This young patient was not subjected to unnecessary chemotherapy. So far only few cases of spontaneous resolution of EBV-associated HLH have been reported.