Fatal Epstein‐Barr virus‐associated hemophagocytic lymphohistiocytosis with virus‐infected T cells after pediatric multivisceral transplantation: A proof‐of‐concept case report

Yamada, Masaki; Sakamoto, Seisuke; Sakamoto, Kenichi; Uchida, Hajime; Shimizu, Seiichi; Osumi, Tomoo; Kato, Motohiro; Shoji, Kensuke; Arai, Katsuhiro; Miyazaki, Osamu; Nakano, Noriyuki; Yoshioka, Takako; Fukuda, Akira; Kasahara, Mureo; Imadome, Ken‐Ichi

doi:10.1111/petr.13961

Cited by 4 publications

(1 citation statement)

References 17 publications

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“…However, EBV-associated HLH manifested worse clinical courses with a 5 year overall survival rate of 33.3% compared to 76% in HLH with unknown causes and infection other than EBV [ 15 ]. As PTLD that is routinely treated with a reduction in immunosuppression resembles EBV-associated HLH, the careful monitoring of proliferation of EBV-infected T cell and consideration of chemotherapy were recommended [ 16 ]. Treatment options in secondary HLH from steroid only to hematopoietic stem cell transplantation (HSCT) or etoposide-based protocol are not organized and still under discussion.…”

Section: Discussionmentioning

confidence: 99%

Hemophagocytic Lymphohistiocytosis as Initial Presentation of Malignancy in Pediatric Patients: Rare but Not to Be Ignored

et al. 2021

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It is complicated to establish a consensus on the management and diagnosis of malignancy-triggered hemophagocytic lymphohistiocytosis (M-HLH) in children, as an initial presentation of malignancy is complicated. In this paper, we analyze the clinical characteristics and outcomes of eight pediatric patients in which M-HLH was the initial presentation of malignancy. All patients had hematologic malignancies: three subcutaneous panniculitis-like T-cell lymphomas, two acute lymphoblastic leukemias, two anaplastic large cell lymphomas, and a systemic EBV + T-cell lymphoma of childhood. The incidence rate of M-HLH among leukemia and malignant lymphoma patients in our institution was 1.9%. From the initial diagnosis of HLH, the median time taken to be diagnosed as a malignancy was about 1.3 months. The majority of patients received HLH-targeted immunosuppression and/or etoposide at first. The patients’ clinical response to treatment for HLH and malignancies were varied. Five out of the eight patients died, one of whom died due to HLH-related cerebral edema after the initiation of chemotherapy. The median overall survival was 1.6 years. In order to improve the survival rate, the early detection of M-HLH, rapid screening for malignancy, and complete control of M-HLH with HLH-directed therapy followed by a thorough response monitoring are required.

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Section: Discussionmentioning

confidence: 99%

Hemophagocytic Lymphohistiocytosis as Initial Presentation of Malignancy in Pediatric Patients: Rare but Not to Be Ignored

et al. 2021

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Hemophagocytic lymphohistiocytosis after solid organ transplantation: A challenge for clinicians

Xu,

2024

Transplant Immunology

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Clinical manifestations and outcomes of EBV related secondary hemophagocytic lymphohistiocytosis

Liu,

Pei,

Zhang

et al. 2024

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Aim: Epstein-Barr virus (EBV) is the important pathogen of infection after allogeneic hematopoietic stem cell transplantation (allo-HSCT), which is also the common cause of secondary hemophagocytic lymphohistiocytosis (sHLH). Thus, we aimed to identify the clinical manifestations and outcomes of EBV-related sHLH after allo-HSCT. Methods: We enrolled the patients who experienced sHLH after EBV DNAemia after allo-HSCT from Jan 1st, 2023 to Dec 31st, 2023. Plasma EBV copies were monitored by Q-PCR analysis at least weekly and EBV-DNA copies of lymphocyte subpopulations in peripheral blood were also quantified by Q-PCR. Results: 11 patients developed sHLH after EBV DNAemia and all of them had post-transplant lymphoproliferative disorders (PTLD) before sHLH. The median time from PTLD to sHLH occurrence was 5 days (range, 2-101) days. The most common manifestation of sHLH was ferritin elevation (100%) and soluble CD25 antigen elevation (100%). Nine (81.8%) patients died after the diagnosis of sHLH, and the median time from the occurrence of sHLH to death was 10 days (range, 3 to 21) days. Temperature ≥ 39.6℃, achieving high-level EBV DNAemia (> 105 copies/ml), and with a high-level EBV in T or NK cells increase the risk of sHLH after EBV-PLTD. The 100-day probability of EBV-related mortality and overall survival after PTLD occurrence was 84.1% versus 8.3 % (P ＜0.0001) and 15.9% versus 91.7% (P ＜0.0001), respectively, for patients with and without sHLH. Conclusions: In summary, our study firstly reported the clinical manifestations and outcomes of EBV-related sHLH following allo-HSCT in the largest cohort at present.

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Fatal Epstein‐Barr virus‐associated hemophagocytic lymphohistiocytosis with virus‐infected T cells after pediatric multivisceral transplantation: A proof‐of‐concept case report

Cited by 4 publications

References 17 publications

Hemophagocytic Lymphohistiocytosis as Initial Presentation of Malignancy in Pediatric Patients: Rare but Not to Be Ignored

Hemophagocytic Lymphohistiocytosis as Initial Presentation of Malignancy in Pediatric Patients: Rare but Not to Be Ignored

Hemophagocytic lymphohistiocytosis after solid organ transplantation: A challenge for clinicians

Clinical manifestations and outcomes of EBV related secondary hemophagocytic lymphohistiocytosis

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