A Case of Severe Epstein–Barr Virus-Associated Hemophagocytic Lymphohistiocytosis Successfully Treated With High Dose Steroids and Ganciclovir

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hematological disorder characterized by immune dysregulation with multiple organ involvement and carries a poor prognosis. The occurrence of HLH can be familial or sporadic, which is triggered by causes like infection or malignancy. This case report is about a 47-year-old male who presented to the hospital with a fever, chills, night sweats, and unintentional weight loss. He was found to have severely elevated ferritin, and computed tomography showed cirrhosis, a normal sized spleen, and retroperitoneal lymphadenopathy. He underwent an extensive battery of tests to identify the etiology. Meanwhile, he had recurrent fevers with worsening transaminitis and septic shock, requiring admission to the ICU. Blood tests for Epstein-Barr virus (EBV) deoxyribonucleic acid (DNA) and immunoglobulin G (IgG) were positive. Due to high suspicion of HLH, he was started on intravenous methylprednisone 1000 mg daily for three days with clinical improvement. A bone marrow biopsy showed hemophagocytosis and he was diagnosed with EBV-associated HLH. He was continued on steroids with oral prednisone and continued to clinically improve. He was later tapered off steroids over the course of five months. HLH is a rapidly progressive and fatal condition that requires prompt treatment, and thus a high index of suspicion is needed to make a timely diagnosis.

Segmental Shielding: A Rare Case of Acute Respiratory Distress Syndrome with Middle Lobe Sparing

“…There is a mechanical ventilator setting recommended by the National Institutes of Health (NIH)-National Heart, Lung, and Blood Institute (NHLBI) ARDS Clinical Network Mechanical Ventilation Protocol; it suggests tidal volume should be 4-8 mL/kg of ideal body weight, respiratory rate up to 35 bpm, SpO2 should be 88-95%, plateau pressure less than 30 cmH2O, pH goal should be 7.30-7.45, and inspiratory-to-expiratory time ratio less than 1. Although the current patient presented with a unique presentation of ARDS, she was given a similar therapeutic approach with the goal of effective lung alveolar recruitment [18][19][20].…”

A therapy that modulates T lymphocyte subsets in patients infected with Epstein-Barr virus: Ganciclovir combined with interferon atomization inhalation

“…[27] Pravash Budhathoki et al successfully treated severe EBV-associated hemophagocytic lymphohistiocytosis with high-dose steroids and ganciclovir. [28] Table 2 Comparison of T lymphocyte subsets between the observation group and the control group before treatment. IFN is an immune-acquired cytokine, which is a group of low molecular weight glycoproteins with similar structure and close function produced by the body's cells through the antiviral response after the body is infected with the virus, and can inhibit the growth of the virus.…”

“…[ 27 ] Pravash Budhathoki et al successfully treated severe EBV-associated hemophagocytic lymphohistiocytosis with high-dose steroids and ganciclovir. [ 28 ]…”