Letter
Oncology
Liang V. Tang, Yu Hu
Summary: This is the first reported case of a healthy individual developing hemophagocytic lymphohistiocytosis (HLH) after receiving the COVID-19 vaccine. The patient's symptoms gradually improved after treatment. The study suggests that excluding the presence of active EBV infection or other common viruses before COVID-19 vaccination is crucial.
JOURNAL OF HEMATOLOGY & ONCOLOGY
(2021)
Article
Immunology
Tingting Cui, Jingshi Wang, Zhao Wang
Summary: This study aimed to build a model for predicting the risk of death during induction therapy in patients with Epstein-Barr virus (EBV)-related hemophagocytic lymphohistiocytosis (HLH). The study included 234 patients with EBV-HLH, of whom 65 (27.4%) died during induction therapy. A nomogram was developed to stratify patients into high-risk and low-risk groups with a high predictive accuracy. The high-risk patients had a significantly higher risk of death compared to the low-risk patients.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Oncology
Yue Song, Jingshi Wang, Yini Wang, Lin Wu, Zhao Wang
Summary: Research has shown that including etoposide in the initial treatment of LAHS can significantly improve remission and survival rates, especially benefiting EBV negative patients.
CANCER BIOLOGY & THERAPY
(2021)
Review
Immunology
Erica A. Steen, Kim E. Nichols, Lauren K. Meyer
Summary: Familial hemophagocytic lymphohistiocytosis (fHLH) is a group of rare inherited immune dysregulation disorders characterized by mutations in genes involved in cytotoxic granules in CD8+ T cells and NK cells. This defect leads to sustained lymphocyte activation and excessive secretion of pro-inflammatory cytokines, resulting in tissue damage and multi-organ failure. Studies in murine models have provided insights into the mechanisms underlying hyperinflammation in fHLH.
FRONTIERS IN IMMUNOLOGY
(2023)
Review
Medicine, General & Internal
Richard Curtis Godby, Ryan R. Kraemer, Jon May, Swati Soni, Vishnu Reddy, John Thomas, Amitkumar Mehta
Summary: This case report presents a rare case of an individual diagnosed with Familial Hemophagocytic Lymphohistiocytosis Type 2 and Chronic Active Epstein-Barr Virus, providing insight into diagnosis and treatment challenges and highlighting the need for further research in this area.
AMERICAN JOURNAL OF THE MEDICAL SCIENCES
(2021)
Article
Obstetrics & Gynecology
Masaya Takahashi, Shintaro Makino, Hiroko Iizuka, Masaaki Noguchi, Koyo Yoshida
Summary: sHLH is a rare and fatal disease that can occur even during pregnancy, characterized by uncontrolled immune cell activation. In this case report, a pregnant patient at 26 weeks gestation presented with symptoms of continuous fever and cytopenia, and after treatment, she recovered but delivered a stillborn baby.
BMC PREGNANCY AND CHILDBIRTH
(2021)
Article
Microbiology
Yan Xie, Li Yang, Pengfei Cao, Shen Li, Wentao Zhang, Wei Dang, Shuyu Xin, Mingjuan Jiang, Yujie Xin, Jing Li, Sijing Long, Yiwei Wang, Senmiao Zhang, Yang Yang, Jianhong Lu
Summary: This study identifies potential biomarkers for the early diagnosis and clinical treatment of EBV-HLH by analyzing the differential plasma exosomal proteome of an EBV-HLH patient and his healthy twin brother. The differentially expressed proteins are related to virus infection, coagulopathy, nervous system dysfunction, imbalance of immune response, and abnormal liver function. These findings provide new insights into the diagnostic and therapeutic biomarkers of EBV-HLH.
FRONTIERS IN MICROBIOLOGY
(2022)
Article
Infectious Diseases
Yubo Pi, Jingshi Wang, Zhao Wang
Summary: We present a case of relapsed EBV-HLH after allo-HSCT that was successfully treated with a PD-1 antibody. Further studies are needed to determine whether PD-1 blockade has therapeutic potential for relapsed EBV-HLH after allo-HSCT.
INFECTION AND DRUG RESISTANCE
(2022)
Article
Allergy
Rebecca A. Marsh, Kyle Hebert, Soyoung Kim, Christopher C. Dvorak, Victor M. Aquino, K. Scott Baker, Deepak Chellapandian, Blachy Davila Saldana, Christine N. Duncan, Michael J. Eckrich, George E. Georges, Timothy S. Olson, Michael A. Pulsipher, Shalini Shenoy, Elizabeth Stenger, Mark Vander Lugt, Lolie C. Yu, Andrew R. Gennery, Mary Eapen
Summary: Allogeneic hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis (HLH) disorders is associated with substantial morbidity and mortality. The study found that conditioning regimens using flu/melphalan and flu/melphalan/thiotepa had better outcomes compared to other regimens in terms of post-transplant complications and survival rates in HLH patients.
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
(2022)
Article
Immunology
Xun Li, Haipeng Yan, Zhenghui Xiao, Ting Luo, Longlong Xie, Yufan Yang, Ling Gong, Zhexuan Tang, Jiaotian Huang, Xinping Zhang, Mincui Zheng, Zhenya Yao, Ping Zang, Desheng Zhu, Xiulan Lu
Summary: The aim of this study was to develop an EBV-HLH screening model for pediatric patients diagnosed with acute infection of EBV, in order to assist in identifying EBV-infected patients who require further evaluation of HLH.
FRONTIERS IN IMMUNOLOGY
(2022)
Review
Medical Laboratory Technology
Tanya Sajan Ponnatt, Cullen M. Lilley, Kamran M. Mirza
Summary: Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening disorder of immune regulation that requires rapid diagnosis and aggressive management. Understanding the pathogenesis and early diagnosis of HLH plays a crucial role in determining patient outcome. HLH can be caused by genetic mutations or acquired factors, and proper management is essential for improving prognosis.
ARCHIVES OF PATHOLOGY & LABORATORY MEDICINE
(2022)
Article
Immunology
Xun Li, Ting Luo, Haipeng Yan, Longlong Xie, Yufan Yang, Ling Gong, Zhexuan Tang, Minghui Tang, Xinping Zhang, Jiaotian Huang, Mincui Zheng, Zhenya Yao, Ping Zang, Desheng Zhu, Zhenghui Xiao, Xiulan Lu
Summary: Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome characterized by immune system activation and excessive proliferation of macrophages and lymphocytes. A proteomic analysis was performed on pediatric HLH patients, revealing alterations in complement and coagulation cascades, neutrophil extracellular trap formation, and platelet activation pathways. The study identified eight differentially expressed proteins, including LCP1 which showed potential as a diagnostic marker for HLH. The findings provide valuable insights and candidate biomarkers for improving understanding of HLH.
JOURNAL OF CLINICAL IMMUNOLOGY
(2023)
Article
Medicine, General & Internal
Xia Lu, Ang Wei, Xu Yang, Jun Liu, Siqi Li, Ying Kan, Wei Wang, Tianyou Wang, Rui Zhang, Jigang Yang
Summary: This study evaluated the role of pre-therapeutic F-18-FDG PET/CT in pediatric HLH with EBV infection. The findings showed that SUVmax-lesions and SUVmax-LN/M performed better in differentiating M-HLH from NM-HLH. The presence of extranodal hypermetabolic lesions in multiple organs indicated the presence of M-HLH. Older age, higher SUVmax-LN and SUVmax-lesions, and the presence of serous effusion were associated with poorer treatment response. SUVmax-lesions and SUVmax-Sp/LiBG might be independent prognostic factors for OS.
FRONTIERS IN MEDICINE
(2022)
Article
Genetics & Heredity
Ruyue Chen, Qiang Lin, Yun Zhu, Yunyan Shen, Qinying Xu, Hanyun Tang, Ningxun Cui, Lu Jiang, Xiaomei Dai, Weiqing Chen, Xiaozhong Li
Summary: The article reports successful cases of using PD-1 inhibitor Sintilimab to treat CAEBV and EBV-HLH. Among the three patients, two had CAEBV and one had EBV-HLH. After treatment, the clinical symptoms of the patients improved significantly, and the EBV-DNA load decreased significantly. The study supports the efficacy of PD-1 targeted therapy in certain patients with CAEBV and EBV-HLH and suggests that Sintilimab may provide a cure without HSCT.
ORPHANET JOURNAL OF RARE DISEASES
(2023)
Article
Hematology
Qingxia Yin, Jingshi Wang, Zhao Wang
Summary: Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is a common subtype of secondary HLH. This study retrospectively analyzed the clinical data of EBV-HLH patients with EBV-CNS infection and found that EBV-CNS infection is an independent risk factor affecting prognosis in these patients.
ANNALS OF HEMATOLOGY
(2022)