Epstein-Barr Virus-Positive Lymphoma-Associated Hemophagocytic Syndrome: A Retrospective, Single-Center Study of 51 Patients

Zhao, Ailin; Yang, Ji; Li, Meng; Li, Linfeng; Gan, Xinai; Wang, Jie; Li, He; Shen, Kai; Yang, Yue; Niu, Ting

doi:10.3389/fimmu.2022.882589

Cited by 7 publications

(3 citation statements)

References 37 publications

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“…Concerning HLH management, we noticed that a majority of patients (53%) required only corticosteroids. In a study that focused on HLH associated with EBV positive lymphoma, only one patient (2.5%) received corticosteroids alone, with a wide use of polychemotherapy [37]. The same rate is reported in another study about mHLH [38].…”

Section: Discussionmentioning

confidence: 53%

Hemophagocytic Lymphohistiocytosis Associated with Immunological Checkpoint Inhibitors: A Pharmacovigilance Study

Diaz

Jauzelon

Dillies

et al. 2023

JCM

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Background: Acquired hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal condition characterized by hyperactivation of macrophages and cytotoxic lymphocytes, combining a series of non-specific clinical symptoms and laboratory disorders. Etiologies are multiple: infectious (mainly viral) but also oncologic, autoimmune or drug-induced. Immune checkpoint inhibitors (ICI) are recent anti-tumor agents associated with a novel profile of adverse events triggered by immune system over-activation. Here, we sought to provide a comprehensive description and analysis of HLH cases reported with ICI since 2014. Methods: Disproportionality analyses were performed in order to further explore the association between ICI therapy and HLH. We selected 190 cases, 177 from the World Health Organization pharmacovigilance database and 13 from the literature. Detailed clinical characteristics were retrieved from the literature and from the French pharmacovigilance database. Results: The cases of HLH reported with ICI concerned men in 65% of cases with a median age of 64 years. HLH occurred in an average of 102 days after the initiation of ICI treatment and mostly concerned nivolumab, pembrolizumab and nivolumab/ipilimumab combination. All cases were considered serious. Most cases presented a favorable outcome (58.4%); however, death was reported for 15.3% of patients. Disproportionality analyses showed that HLH was seven times more frequently reported with ICI therapy than with other drugs and three times more than with other antineoplastic agents. Conclusions: Clinicians should be aware of the potential risk of ICI-related HLH to improve the early diagnosis of this rare immune-related adverse event.

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Section: Discussionmentioning

confidence: 53%

Hemophagocytic Lymphohistiocytosis Associated with Immunological Checkpoint Inhibitors: A Pharmacovigilance Study

Diaz

Jauzelon

Dillies

et al. 2023

JCM

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“…Here, we are focusing on virus-associated haemophagocytic syndrome (VAHS). Recent research has shown that several types of viruses, including EBV [32,33], CMV [34], HIV [35], parainfluenza virus [36], human herpesvirus Type 6 (HHV-6) [37] and other types of viruses [38] can trigger HLH. Among VAHS, EBV is major cause of HLH, and EBV-HLH is associated with a high risk of death and multi-organ failure.…”

Section: Virus-associated Haemophagocytic Syndromementioning

confidence: 99%

New sights of low dose IL‐2: Restoration of immune homeostasis for viral infection

Su,

Zhang,

Wang

et al. 2023

Immunology

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Viral infection poses a significant threat to human health. In addition to the damage caused by viral replication, the immune response it triggers often leads to more serious adverse consequences. After the occurrence of viral infection, in addition to the adverse consequences of infection, chronic infections can also lead to virus‐related autoimmune diseases and tumours. At the same time, the immune response triggered by viral infection is complex, and dysregulated immune response may lead to the occurrence of immune pathology and macrophage activation syndrome. In addition, it may cause secondary immune suppression, especially in patients with compromised immune system, which could lead to the occurrence of secondary infections by other pathogens. This can often result in more severe clinical outcomes. Therefore, regarding the treatment of viral infections, restoring the balance of the immune system is crucial in addition to specific antiviral medications. In recent years, scientists have made an interesting finding that low dose IL‐2 (ld‐IL‐2) could potentially have a crucial function in regulating the immune system and reducing the chances of infection, especially viral infection. Ld‐IL‐2 exerts immune regulatory effects in different types of viral infections by modulating CD4+T subsets, CD8+T cells, natural killer cells, and so on. Our review summarised the role of IL‐2 or IL‐2 complexes in viral infections. Ld‐IL‐2 may be an effective strategy for enhancing host antiviral immunity and preventing infection from becoming chronic; additionally, the appropriate use of it can help prevent excessive inflammatory response after infection. In the long term, it may reduce the occurrence of infection‐related autoimmune diseases and tumours by promoting the restoration of early immune homeostasis. Furthermore, we have also summarised the application of ld‐IL‐2 in the context of autoimmune diseases combined with viral infections; it may be a safe and effective strategy for restoring immune homeostasis without compromising the antiviral immune response. In conclusion, focusing on the role of ld‐IL‐2 in viral infections may provide a new perspective for regulating immune responses following viral infections and improving prognosis.

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“…Hemophagocytic syndromes (HPS), also known as hemophagocytic lymphohistiocytosis (HLH), is a syndrome characterized by excessive immune activation and is often characterized by fever, hepatosplenomegaly, and complete blood cell Patients with HLH are initially similar to viral colds, often with fever as the first symptom, but the disease progresses rapidly and is prone to multi-organ involvement, leading to progressive multi-organ failure, triple cytopenia and multi-organ damage to the lungs, liver and spleen in acute and severe cases, with a high mortality [3][4]. According to the cause of onset HLH can be divided into primary HLH and secondary HLH [5], the former is characterized by early-onset and cytotoxicity-related genetic mutations, a clear family history of the disease, and a possible association with inherited genetic defects, with childhood onset more often than adult; the latter is mainly secondary to malignancies, infections, autoimmune diseases, and connective tissue diseases, among which EBV-associated HLH ( EBV-HLH) is the most common type of viral secondary HLH and is a life-threatening hyperinflammatory response syndrome with an aggressive prognosis, high mortality rate, easy relapse and shorter survival time [6][7][8].…”

Section: Western Medical Pathogenesismentioning

confidence: 99%

A Case of EBV-associated Phagocytic Syndrome Treated on the Basis of the Academic Idea of Five Elements of Qi-hua Theory

2022

IJPHPM

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Hemophagocytic syndrome (HPS), also known as hemophagocytic lymphohistiocytosis (HLH), is a rare and critical clinical syndrome. It is a rare and critical clinical syndrome with rapid onset, rapid progression, and a high mortality rate. At present, western medical treatment of HLH is mainly based on the diagnostic and therapeutic protocols of HLH-1994 or HLH-2004: control of excessive inflammation, delaying the progression of HLH activation; treatment of the primary cause of the disease and immunotherapy. In this paper, based on Liu Huawei's academic thought of "five elements and qi-hua theory", combined with the dialectical theory of "Wei-Qi-Ying-Blood" in Chinese medicine, we treated a case of HLH with Chinese medicine and achieved good clinical efficacy. The successful diagnosis and treatment of this patient provides some implications for the clinical use of TCM in the treatment of rare infectious diseases and hematological diseases.

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Epstein-Barr Virus-Positive Lymphoma-Associated Hemophagocytic Syndrome: A Retrospective, Single-Center Study of 51 Patients

Cited by 7 publications

References 37 publications

Hemophagocytic Lymphohistiocytosis Associated with Immunological Checkpoint Inhibitors: A Pharmacovigilance Study

Hemophagocytic Lymphohistiocytosis Associated with Immunological Checkpoint Inhibitors: A Pharmacovigilance Study

New sights of low dose IL‐2: Restoration of immune homeostasis for viral infection

A Case of EBV-associated Phagocytic Syndrome Treated on the Basis of the Academic Idea of Five Elements of Qi-hua Theory

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