Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in adults and adolescents—a life-threatening disease: analysis of 133 cases from a single center

Lai, Wenyuan; Wang, Yini; Wang, Jingshi; Lin, Wei; Jin, Zhili; Wang, Zhao

doi:10.1080/10245332.2018.1491093

Cited by 42 publications

(45 citation statements)

References 25 publications

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“…In one Japanese cohort, allo-HSCT resulted in an 85.7% 10-year OS for patients with EBV-HLH [20]. In a retrospective analysis from our centre, 27.1% of patients with EBV-HLH received allo-HSCT, and the nal survival rate was 52.78% [21]. However, some patients in the actual situation are unable to receive allo-HCST due to various factors.…”

Section: Discussionmentioning

confidence: 92%

“…Among the infections-associated HLH, Epstein-Barr virus (EBV) infection-associated HLH (EBV-HLH) is one of the most common. In previous studies, EBV-HLH patients suffered a much worse prognosis than patients with other types of infection-associated HLH, especially in adult HLH patients [3][4][5]. In 2015, a study of 61 cases with EBV-HLH reported a 1-year overall survival (OS) of only 25.0% [6].…”

Section: Introductionmentioning

confidence: 99%

“…The current rst-line treatment regimen, HLH-94 followed by allo-HSCT fails to trigger a response under ideal conditions in 30% of children with all HLH triggers [7]. In a study of 133 adults and adolescents with EBV-HLH, the non-response rate of the HLH-94 regimen was 52% [5]. The DEP (doxorubicin-etoposide-methylprednisolone) regimen and L-DEP (PEG-asparaginase and DEP regimen combination) regimen, which is used as a salvage therapy for refractory EBV-HLH, achieves a much better overall response rate and increase the chance to receive allo-HSCT, which improves survival [8][9][10].…”

Section: Introductionmentioning

confidence: 99%

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HLA-Mismatched GPBSCs infusion therapy in refractory Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis: A observational study from a single center

Song

Wang

et al. 2020

Preprint

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Abstract Background: Hemophagocytic lymphohistiocytosis (HLH) is a severe or even fatal inflammatory state. Epstein–Barr virus (EBV) infection associated HLH (EBV-HLH) is one of the most common secondary HLH and suffers a very poor prognosis. Allo-HSCT is often required for refractory EBV-HLH, but some patients still cannot proceed to the next allo-HSCT due to various factors. This study aimed to observe the efficacy of HLA-mismatched granulocyte colony-stimulating factor (G-CSF) mobilized peripheral blood stem cells (GPBSCs) infusion for refractory EBV-HLH. Methods: A retrospective case-control study of refractory EBV-HLH patients with GPBSCs infusion from HLA-mismatched donors after chemotherapy (as GPBSCs group) and sole chemotherapy (as control group) was performed. Efficacy was evaluated 2 and 4 weeks and all patients were followed up until 1 March 2018.Results: There were 18 cases who accepted infusion between March 2016 and Sep 2017 and 19 were randomly selected from refractory EBV-HLH patients who underwent salvage therapy during the same period for the control group. In GPBSCs group, WBC (p=0.017), Fbg (p=0.040), ferritin (p=0.039) improved significantly after treatment. The overall response rate was 66.7% (CR 22.2%, PR 44.4%). However, there is no significant differences in changes of WBC, HGB, PLT, TG, Fbg, Ferritin, AST, ALT, T-bil between two groups. Only the Fbg level was recovered better in the GPBSCs infusion group (p=0.003). In the GPBSCs group, EBV-DNA decreased significantly after 2 weeks (p=0.001) and 4 weeks (p=0.012) after treatment, and the effect of the decrease was significantly better than that of the chemotherapy alone group in 2 weeks but not 4 weeks (p2w=0.011, p4w=0.145). The median survival time in the infusion group was 20.4 weeks [95%CI 10.9, 29.9], and the median survival time in the control group was 10.8 weeks [95%CI 0-24.34]. In the short-term, the infusion group’s survival rate was better (2-month 88.89% vs. 52.63%, p=0.008; 3-month 83.33% vs. 47.09%, p=0.012), but there was no difference in OS (p=0.287). Conclusions: Infusing GPBSCs combined with chemotherapy is effective, especially in decreasing EBV-DNA, performs better than chemotherapy alone, and improve short term survival rate. GPBSCs infusion is suggested as a bridging treatment method to allo-HSCT.

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Section: Discussionmentioning

confidence: 92%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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HLA-Mismatched GPBSCs infusion therapy in refractory Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis: A observational study from a single center

Song

Wang

et al. 2020

Preprint

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“…will not respond to HLH-94 [7]. In a study of adults and adolescents EBV-HLH 133 patients, the nonresponse rate of HLH-94 regimen is 52% [5]. Our center's DEP (doxorubicin-etoposidemethylprednisolone) regimen and L-DEP (PEG-aspargase and DEP regimen combination) as the salvage therapy for refractory EBV-HLH achieve a much better overall response rate and increase the chance to receive allo-HSCT, which improves survival [8][9][10].…”

Section: Introductionmentioning

confidence: 96%

“…Among the infection associated HLH, Epstein-Barr virus (EBV) infection associated HLH (EBV-HLH) is one of the most common. In previous studies, EBV-HLH patients suffer a much worse prognosis than other type of infection associated HLH, especially in adult HLH patients [3][4][5]. A previous study of 61 cases of EBV-HLH in 2015, reported a 1year overall survival (OS) of only 25.0% [6].…”

Section: Introductionmentioning

confidence: 99%

HLA-Mismatched GPBSCs infusion therapy in refractory Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis: A observational study from a single center

Song

Wang

et al. 2020

Preprint

Self Cite

View full text Add to dashboard Cite

Abstract Background: Hemophagocytic lymphohistiocytosis (HLH) is a severe or even fatal inflammatory state. Epstein–Barr virus (EBV) infection associated HLH (EBV-HLH) is one of the most common secondary HLH and suffers a very poor prognosis. Allo-HSCT is often required for refractory EBV-HLH, but some patients still cannot proceed to the next allo-HSCT due to various factors. This study aimed to observe the efficacy of HLA-mismatched granulocyte colony-stimulating factor (G-CSF) mobilized peripheral blood stem cells (GPBSCs) infusion for refractory EBV-HLH. Methods: A retrospective case-control study of refractory EBV-HLH patients with GPBSCs infusion from HLA-mismatched donors after chemotherapy (as GPBSCs group) and sole chemotherapy (as control group) was performed. Efficacy was evaluated 2 and 4 weeks and all patients were followed up until 1 January 2018. Results: There were 15 cases who accepted infusion between March 2016 and June 2017 and 19 were randomly selected from refractory EBV-HLH patients who underwent salvage therapy during the same period for the control group. In GPBSCs group, WBC (p=0.029), Fbg (p=0.041), ferritin (p=0.041) improved significantly after treatment. The overall response rate was 66.7% (CR 26.7%, PR 40.0%). However, there is no significant differences in changes of WBC, HGB, PLT, TG, Fbg, Ferritin, AST, ALT, T-bil between two groups. Only the Fbg level was recovered better in the GPBSCs infusion group (p=0.017). In the GPBSCs group, EBV-DNA decreased significantly after 2 weeks (p=0.010) and 4 weeks (p=0.002) after treatment, and the effect of the decrease was significantly better than that of the chemotherapy alone group in 2 weeks but not 4 weeks (p2w=0.017, p4w=0.145). The median survival time in the infusion group was 20.4 weeks [6.7, 92.1], and the median survival time in the control group was 10.8 weeks [0.6-116.4]. In the short-term, the infusion group’s survival rate was better (2-month 93.33% vs. 52.63%, p=0.002; 3-month 86.67% vs. 47.09%, p=0.006), but there was no difference in OS (p=0.259). Conclusions: Infusing GPBSCs combined with chemotherapy is effective, especially in decreasing EBV-DNA, performs better than chemotherapy alone, and improve short term survival rate. GPBSCs infusion is suggested as a bridging treatment method to allo-HSCT.

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Acute liver injury secondary to hemophagocytic lymphohistiocytosis triggered by Epstein–Barr virus infection

et al. 2020

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Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in adults and adolescents—a life-threatening disease: analysis of 133 cases from a single center

Abstract: DEP/L-DEP was a good salvage treatment. L-DEP might be a more effective first-line initial regimen than HLH-94/04 regimen for EBV-HLH. Finally, allo-HSCT is an effective radical treatment for EBV-HLH.

Cited by 42 publications

References 25 publications

HLA-Mismatched GPBSCs infusion therapy in refractory Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis: A observational study from a single center

HLA-Mismatched GPBSCs infusion therapy in refractory Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis: A observational study from a single center

HLA-Mismatched GPBSCs infusion therapy in refractory Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis: A observational study from a single center

Acute liver injury secondary to hemophagocytic lymphohistiocytosis triggered by Epstein–Barr virus infection

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