Treatment of Epstein‐Barr virus‐associated hemophagocytic lymphohistiocytosis (EBV‐HLH) in young adults: A report from the HLH studyl center

Imashuku, Shinsaku; Kuriyama, Kikuko; Sakai, Rika; Nakao, Yoshitaka; Masuda, Saeko; Yasuda, Norimasa; Kawano, Fumio; Yakushijin, Kimikazu; Miyagawa, Akiko; Nakao, Taisei; Teramura, Tomoko; Tabata, Yasuhiro; Morimoto, Akira; Hibi, Shigeyoshi

doi:10.1002/mpo.10314

Cited by 134 publications

(82 citation statements)

References 31 publications

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“…5 In young adult patients, Imashuku et al reported that treatment with etoposide within four weeks of diagnosis was extremely effective in improving the outcome of EBV-HLH. 6 In the current case, we could not control hypercytokinemia despite the multiple combination therapy.…”

Section: Discussionmentioning

confidence: 62%

Cytokine nephropathy in a patient with fatal Epstein–Barr virus-associated hemophagocytic syndrome

2013

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Occasionally, patients with acute Epstein-Barr virus (EBV) infection develop hemophagocytic syndrome (HPS). Acute kidney injury (AKI) is considered a strong prognostic factor, but very few data are available about the biopsy-proven renal involvement of EBV-HPS. Here we describe a previously healthy 17-year-old girl with EBV-HPS. Combination therapy failed and renal necropsy was performed. The renal histology showed that intact glomeruli, remarkable interstitial edema and some cellular infiltration, and protein casts. These findings were compatible with cytokine nephropathy as recently advocated. We suggest that hypercytokinemia may play an important role in the pathophysiology in AKI of EBV-HPS.

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Section: Discussionmentioning

confidence: 62%

Cytokine nephropathy in a patient with fatal Epstein–Barr virus-associated hemophagocytic syndrome

2013

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“…However, in some cases of HLH, the initiating cause is not identified. Because of its heterogeneous etiology and nonspecific clinical and laboratory findings which often mimic other conditions like sepsis, the diagnosis of secondary HLH may be delayed or unrecognized [12,13].…”

Section: Introductionmentioning

confidence: 99%

Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis

2015

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Hemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome characterized by the activation of the mononuclear phagocytic system. The diagnosis of HLH in adults is challenging not only because the majority of the reported data are from pediatric patients, but also because HLH occurs in many disease entities. This study reports the clinical and laboratory findings and prognostic factors of adult HLH in a large cohort managed at a single medical center from 2003 to 2014. Seventy-three patients met the HLH-2004 diagnostic criteria. The median age was 51 years (range, 18-82 years); 41 (56.2%) were male. Patients manifested fever, cytopenias, and elevated ferritin in >85% of cases. Likely causes of HLH were as follows: 30 (41.1%) infections, 21 (28.8%) malignancies, 5 (6.8%) attributed to autoimmune disorders, 1 (1.4%) primary immunodeficiency, 2 (2.7%) post solid organ transplantation, and 13 (17.8%) idiopathic. The median overall survival was 7.67 months. Patients with malignancy-associated HLH had a markedly worse survival compared with patients with non-malignancy-associated HLH (median overall survival 1.13 vs. 46.53 months, respectively; P < 0.0001). In a multivariable analysis, malignancy (hazard ratio 5 12.22; 95% CI: 2.53-59.02; P 5 0.002) correlated with poor survival. Ferritin >50,000 mg/L correlated with 30-day mortality. Survival after a diagnosis of HLH is dismal, especially among those with malignancy-associated HLH. The development of a registry for adults with HLH would improve our understanding of this syndrome, validate diagnostic criteria, and help develop effective treatment strategies.

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“…All current studies on the treatment of adults are noncontrolled and retrospective small-sample studies, 16,[24][25][26] and this study provides encouraging results for the salvage therapy of refractory HLH. Our study might bridge the transition from induction therapy to etiology therapy.…”

Section: Discussionmentioning

confidence: 69%

Multicenter study of combination DEP regimen as a salvage therapy for adult refractory hemophagocytic lymphohistiocytosis

Wang

Huang

et al. 2015

Blood

141

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Key Points• A salvage therapy for adults with refractory hemophagocytic lymphohistiocytosis.• Liposomal doxorubicin treatment combined with etoposide and methylprednisolone showed an encouraging overall response and was welltolerated.Hemophagocytic lymphohistiocytosis (HLH) is a refractory immune disorder with a significant risk of death. Although standard therapy has dramatically improved survival in HLH patients, approximately 30%, especially adults, show no response to current treatment strategies. This prospective study aimed to investigate the efficacy of liposomal doxorubicin treatment combined with etoposide and methylprednisolone (doxorubicin-etoposidemethylprednisolone; DEP) as a salvage therapy for adult refractory HLH. Adult patients who did not achieve at least partial response 2 weeks after initial standard HLH therapy were enrolled in this study between June 2013 and June 2014. Response to salvage therapy was assessed at 2 and 4 weeks after initiation of DEP therapy and patients were followed until death or until November 2014. Sixty-three refractory HLH patients were enrolled, including 29 cases of lymphoma-associated HLH, 22 cases of Epstein-Barr virus-associated HLH, and 4 cases of familial HLH. There were 8 cases with unknown underlying diseases. Seventeen cases (27.0%) achieved complete response and 31 cases (49.2%) achieved partial response. The overall response was 76.2% (48/63). Patients who showed no response to DEP died within 4 weeks after salvage therapy. Twenty-nine of the 48 patients who achieved partial or complete response survived to subsequent chemotherapy, allogenic hematopoietic stem cell transplantation, or splenectomy. Our study suggests that DEP regimen is an effective salvage regimen for adult refractory HLH, which can prolong patient survival as we continue to understand the responsible mechanisms and bridge the gap between HLH and its underlying diseases. This study was registered in the Chinese Clinical Trials Registry Platform

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Treatment of Epstein‐Barr virus‐associated hemophagocytic lymphohistiocytosis (EBV‐HLH) in young adults: A report from the HLH studyl center

Cited by 134 publications

References 31 publications

Cytokine nephropathy in a patient with fatal Epstein–Barr virus-associated hemophagocytic syndrome

Cytokine nephropathy in a patient with fatal Epstein–Barr virus-associated hemophagocytic syndrome

Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis

Multicenter study of combination DEP regimen as a salvage therapy for adult refractory hemophagocytic lymphohistiocytosis

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