Combining an antiviral with rituximab in EBV-related haemophagocytic lymphohistiocytosis led to rapid viral clearance; and a comprehensive review

Stefanou, Christos; Tzortzi, Christiana; Georgiou, Fotini; Timiliotou, Chrystalla

doi:10.1136/bcr-2016-216488

Cited by 10 publications

(5 citation statements)

References 69 publications

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“…Antivirals are insufficient for EBV clearance, and there is no established best antiviral method. 22,23 Previous studies have revealed that rituximab can successfully reduce viral load in EBV-infected B cell-related HLH in Western countries. 22,24 However, EBV infection was predominantly found in T cells and/or natural killer cells in patients with HLH from China mainland and other East Asia areas.…”

Section: Resultsmentioning

confidence: 99%

Early Use of Blood Purification in Severe Epstein-Barr Virus–Associated Hemophagocytic Syndrome

Huang

et al. 2020

Pediatrics

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Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is a common type of hemophagocytic lymphohistiocytosis (HLH) that exhibits high rates of morbidity and fatalities. Multiorgan failure caused by Epstein-Barr virus (EBV)-induced hypercytokinemia is one of the main reasons for early deaths. Blood purification techniques have been successfully applied in previously treated hypercytokinemia. However, there were insufficient studies to support the combination of plasma exchange (PE) and continuous renal replacement therapy (CRRT) in treating patients with severe EBV-HLH. In this article, we have summarized the effects of early incorporation of PE and CRRT, together with HLH-2004 chemoimmunotherapy, in 8 pediatric patients with severe EBV-HLH. Early use of PE and CRRT appeared to be well tolerated, and no serious side effects and early deaths were observed. After PE and CRRT procedures, cytokine levels were reduced to normal values, except for soluble interleukin 2 receptor, and significant reductions in EBV DNA, serum ferritin, aspartate transaminase, total bilirubin, total bile acid, lactate dehydrogenase, and body temperature values and increases in the neutrophil count in addition to hemoglobin, albumin, and cholinesterase values were observed. Furthermore, through continuous HLH-2004 treatment regimens, lower limits of detection were exhibited for EBV DNA levels, and all other observational indicator levels were restored to normal. Finally, 7 patients achieved and maintained complete remission for 15 to 24 months, culminating in August 2019. Therefore, it is our suggestion that early incorporation of PE and CRRT with chemoimmunotherapy might be a safe and effective treatment for patients with severe EBV-HLH.Hemophagocytic syndrome, or hemophagocytic lymphohistiocytosis (HLH), is a life-threatening disease. Epstein-Barr virus (EBV) is one of the most common triggers for HLH, particularly in Asian countries. 1,2 The recently reported long-term overall survival rate of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) in children ranges from 43.3% to 91.2%, [3][4][5][6] and the control of early death is still a challenging issue. [7][8][9] More than half of all deaths occurred within 30 days after diagnosis. 9,10 Hypercytokinemia-induced multiorgan failure is one of the main reasons for early death in EBV-HLH. 11 Extracorporeal blood purification techniques have been successfully applied to normalize serum cytokine levels in sepsis and septic shock, 12 which, in some respects, are similar to

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Section: Resultsmentioning

confidence: 99%

Early Use of Blood Purification in Severe Epstein-Barr Virus–Associated Hemophagocytic Syndrome

Huang

et al. 2020

Pediatrics

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“…In our patient who is medically stable, we have elected to pursue a dexamethasone induction course while monitoring response to therapy. Besides plasma exchange and intravenous immunoglobulin, emerging therapies that warrant further clinical investigation include anti-IL-1, anti-TNF-α, anti-IFN-γ treatments [32] as well as kinase inhibitors [32], rituximab [38] and anti-virals [39].…”

Section: Discussionmentioning

confidence: 99%

Hemophagocytic Lymphohistiocytosis Caused by a Severe Epstein-Barr Virus Infection in a Young Patient Presenting With Hiccups

Kantono¹,

Bhagat²,

Hu³

et al. 2023

Cureus

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Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome characterized by a pathologic immune response in the setting of infection, malignancy, acute illness, or any immunological stimulus. Infection is the most common etiology of HLH. HLH involves aberrant activation of lymphocytes and macrophages with resultant hypercytokinemia due to an inappropriately stimulated and ineffective immune response. Here, we present the case of a previously healthy 19-year-old male presenting with hiccups and scleral icterus, who was found to have HLH due to a severe Epstein-Barr virus infection. Despite a morphologically normal bone marrow biopsy, the patient met the diagnostic criteria for HLH, including a low natural killer cell count and elevated soluble interleukin-2 receptor. Notably, ferritin was severely elevated at 85,810 ng/mL. The patient was treated with an induction course of dexamethasone intravenously for eight weeks. Since HLH can progress into multi-organ failure, timely diagnosis and prompt initiation of treatment are critical. Novel disease-modifying therapies and further clinical trials are warranted to treat this potentially fatal immunological disease with multisystem ramifications.

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“…Multiple studies on the effects of different antiviral drugs on EBV have been conducted. Therefore, the importance of finding an effective antiviral drug or vaccine becomes increasingly evident [40, 42].…”

Section: Discussionmentioning

confidence: 99%

Detection of Major Human Herpesviruses in Iranian Patients with Suspected Encephalitis Using Multiplex Polymerase Chain Reaction

et al. 2021

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Background: The Herpesviridae family plays a significant etiological role in central nervous system viral infections during primary infection or reactivation from a latent form. Early detection is crucial because prescribing some antivirals can prevent severe side effects or life-threatening conditions. Methods: In this study, 251 CSF specimens were collected from patients with clinical suspicion of viral encephalitis in Pars Hospital, Tehran, Iran. DNA was extracted, and a multiplex PCR was designed to investigate the presence of herpes simplex virus-1, herpes simplex virus-2, varicella zoster virus, Epstein-Barr virus, and cytomegalovirus. Results: Overall, 59 cases of the 251 CSF samples were positive for multiplex PCR (23.5%). The most frequent positive findings were EBV and HSV, with a prevalence of 10.3% and 8.7% (5.5% HSV-1 and 3.1% HSV-2), respectively. Four co-infections were also seen in this study. Conclusions: This multiplex PCR assay detects simultaneously different herpesviruses in CSF samples of patients with suspected encephalitis in 2 rounds of PCR amplification; therefore, it is a reliable and cost-saving diagnostic method for evaluating patients infected with herpesvirus with neurological disorders.

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Combining an antiviral with rituximab in EBV-related haemophagocytic lymphohistiocytosis led to rapid viral clearance; and a comprehensive review

Cited by 10 publications

References 69 publications

Early Use of Blood Purification in Severe Epstein-Barr Virus–Associated Hemophagocytic Syndrome

Early Use of Blood Purification in Severe Epstein-Barr Virus–Associated Hemophagocytic Syndrome

Hemophagocytic Lymphohistiocytosis Caused by a Severe Epstein-Barr Virus Infection in a Young Patient Presenting With Hiccups

Detection of Major Human Herpesviruses in Iranian Patients with Suspected Encephalitis Using Multiplex Polymerase Chain Reaction

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