Review
Hematology
Chenzi Zhao, Qing Zhang, Rui Zhang, Hongyun Lian, Honghao Ma, Xiaoxi Zhao, Zhigang Li
Summary: This study analyzed the genetic variation and prognosis of primary hemophagocytic lymphohistiocytosis (pHLH) in children and the clinical features of isolated central nervous system HLH (CNS-HLH). The most common gene variants in pHLH patients were UNC13D, PRF1, and XIAP. CNS involvement was higher in pHLH patients compared to secondary HLH. Isolated CNS-HLH patients are often misdiagnosed, and hematopoietic stem cell transplantation (HSCT) may be beneficial for these patients. NK cell cytotoxicity and CD107a levels were not accurate in distinguishing pHLH from secondary HLH.
ANNALS OF HEMATOLOGY
(2023)
Review
Pediatrics
Shinsaku Imashuku, Akira Morimoto, Eiichi Ishii
Summary: HLH can be categorized as primary and secondary, with viral HLH being the major type across all age groups. Different types of HLH require specific treatment strategies, such as using immunomodulatory agents for EBV-HLH and more aggressive therapies for refractory cases.
Review
Immunology
Aurora Chinnici, Linda Beneforti, Francesco Pegoraro, Irene Trambusti, Annalisa Tondo, Claudio Favre, Maria Luisa Coniglio, Elena Sieni
Summary: Hemophagocytic Lymphohistiocytosis (HLH) is a rare clinical condition characterized by sustained but ineffective immune system activation, leading to severe and systemic hyperinflammation. Prompt diagnosis and treatment are crucial for survival, as a considerable proportion of patients with HLH still die from progressive disease. Expert consultation and genetic analysis are recommended for accurate interpretation and therapeutic decisions.
FRONTIERS IN IMMUNOLOGY
(2023)
Review
Medicine, Research & Experimental
Chiara Benevenuta, Ilaria Mussinatto, Cecilia Orsi, Fabio S. S. Timeus
Summary: Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening condition characterized by uncontrolled and ineffective immune response, leading to hyperinflammation. This review focuses on secondary HLH in pediatric patients, discussing its epidemiology, pathophysiology, diagnosis, treatment, prognosis, as well as subtypes and new insights in diagnosis and treatment.
EXPERIMENTAL AND THERAPEUTIC MEDICINE
(2023)
Article
Hematology
Qunxiang Qiu, Dan Li, Ying Chen, Ying Song, Shifeng Lou, Kang Zhou, Jianchuan Deng
Summary: We analyzed the clinical features and prognostic risk factors of adult HLH and developed a prognostic model to predict the overall survival of adult HLH. IL-10 and PT were identified as independent factors affecting the survival rate. A prognostic model consisting of IL-2R, PT, and IL-10 was developed, which showed high prediction accuracy for 1-year, 2-year, and 3-year survival rates.
ANNALS OF HEMATOLOGY
(2023)
Article
Public, Environmental & Occupational Health
Geydson Silveira Cruz, Amelia Ribeiro de Jesus, Roque Pacheco Almeida, Maria Aurelia Porto
Summary: Visceral leishmaniasis (VL) is a neglected tropical disease with high mortality risk and diverse clinical presentations. We reported three cases of VL with bone marrow hemophagocytosis and HLH.
AMERICAN JOURNAL OF TROPICAL MEDICINE AND HYGIENE
(2021)
Article
Immunology
Stephanie J. Si, Sarah K. Tasian, Hamid Bassiri, Brian T. Fisher, Jasmyn Atalla, Reema Patel, Neil Romberg, Michele P. Lambert, Michele Paessler, Edward J. Behrens, David T. Teachey, Kathleen E. Sullivan
Summary: HLH is a syndrome of severe immune dysregulation with a complex diagnosis process, leading to potential delays in treatment initiation.
JOURNAL OF CLINICAL IMMUNOLOGY
(2021)
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
Snehal Shabrish, Madhura Kelkar, Reetika Malik Yadav, Umair Ahmed Bargir, Maya Gupta, Aparna Dalvi, Jahnavi Aluri, Manasi Kulkarni, Shweta Shinde, Sneha Sawant-Desai, Priyanka Kambli, Gouri Hule, Priyanka Setia, Neha Jodhawat, Pallavi Gaikwad, Amruta Dhawale, Nayana Nambiar, Vijaya Gowri, Ambreen Pandrowala, Prasad Taur, Revathi Raj, Ramya Uppuluri, Ratna Sharma, Pranoti Kini, Meena Sivasankaran, Deenadayalan Munirathnam, Ramprasad Vedam, Pandiarajan Vignesh, Aaqib Banday, Amit Rawat, Amita Aggarwal, Ujjal Poddar, Meenakshi Girish, Abhijit Chaudhary, Abhilasha Sampagar, Dharani Jayaraman, Narendra Chaudhary, Nitin Shah, Farah Jijina, S. Chandrakla, Swati Kanakia, Brijesh Arora, Santanu Sen, Madhukar Lokeshwar, Mukesh Desai, Manisha Madkaikar
Summary: Hemophagocytic lymphohistiocytosis (HLH) is characterized by immune dysregulation and systemic inflammation. In India, FHL2 and FHL3 are the most common subtypes. Genetic heterogeneity in the Indian population and poor prognosis of FHL were confirmed in this study, emphasizing the importance of flow cytometry-based assays in rapid diagnosis and validation of novel variants.
FRONTIERS IN IMMUNOLOGY
(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)
Article
Immunology
Kristina E. N. Clark, William D. Nevin, Tabitha Mahungu, Helen Lachmann, Animesh Singh
Summary: This study found similarities in the clinical manifestations and molecular inflammatory responses between COVID-19 patients and HLH patients, but did not support the use of HLH risk-scoring methodology for risk stratification in COVID-19 patients.
CLINICAL INFECTIOUS DISEASES
(2021)
Article
Pediatrics
Niveditha Balakumar, Prithvi Sendi, Balagangadhar R. Totapally
Summary: This study describes the prevalence, clinical characteristics, interventions, and outcomes of neonates diagnosed with hemophagocytic lymphohistiocytosis (HLH) in the United States. It found that HLH is a rare diagnosis with a high mortality rate in neonates. Herpes simplex virus is the most common infection associated with neonatal HLH, and HLH should be considered in neonates presenting with multi-organ dysfunction or sepsis.
FRONTIERS IN PEDIATRICS
(2022)
Review
Pathology
Leonard Naymagon
Summary: Originally designed for pediatric HLH, the HLH-2004 criteria have been used to diagnose adult secondary HLH, raising questions about their applicability. Current evidence is lacking on the use of these criteria in adult secondary HLH cases.
PATHOLOGY RESEARCH AND PRACTICE
(2021)
Article
Medicine, General & Internal
Thomas El Jammal, Arthur Guerber, Martin Prodel, Maxime Fauter, Pascal Seve, Yvan Jamilloux
Summary: This study proposes a machine learning approach to build a classification model for HLH in a cohort of patients selected by glycosylated ferritin dosage in a tertiary center in Lyon, France. The model showed good diagnostic performances on a dataset of 207 adult patients, but further studies are needed to improve its performance.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Medical Laboratory Technology
Zhili Jin, Dina Suolitiken, Yini Wang, Zhao Wang
Summary: This study suggests that multiple cytokines can serve as valid biological markers for the diagnosis of HLH, with IL-18, IL-1 RA, and IP-10 showing high diagnostic accuracy. Further validation in external datasets is warranted.
JOURNAL OF CLINICAL LABORATORY ANALYSIS
(2023)
Editorial Material
Chemistry, Multidisciplinary
Lu Tie, Han Xiao, Da-lei Wu, Ye Yang, Pei Wang
ACTA PHARMACOLOGICA SINICA
(2021)
Letter
Oncology
Chunyan Gu, Ting Lu, Wang Wang, Miaomiao Shao, Rongfang Wei, Mengjie Guo, Rui Li, Li Qiao, Ye Hu, Fenghuang Zhan, Anja Seckinger, Dirk Hose, Ye Yang
Review
Biochemistry & Molecular Biology
Xiaozhu Tang, Hongyan Ren, Mengjie Guo, Jinjun Qian, Ye Yang, Chunyan Gu
Summary: circRNAs are a class of interesting and conserved single-stranded RNA molecules that play important roles in various biological processes, especially in microRNA sponges, gene splicing and transcription regulation, RNA-binding protein sponges, and protein/peptide translation. They are involved in various human diseases, particularly cancers, and may serve as better predictive biomarkers and therapeutic targets for cancer treatment.
COMPUTATIONAL AND STRUCTURAL BIOTECHNOLOGY JOURNAL
(2021)
Article
Cell Biology
Renjie Dou, Jinjun Qian, Wei Wu, Yanxin Zhang, Yuxia Yuan, Mengjie Guo, Rongfang Wei, Shu Yang, Artur Jurczyszyn, Siegfried Janz, Meral Beksac, Chunyan Gu, Ye Yang
Summary: SRD5A1 downregulation simultaneously regulates both the apoptosis and the autophagic process in multiple myeloma, serving as a potential biomarker and target for disease progression and prognosis.
CELL DEATH & DISEASE
(2021)
Letter
Oncology
Fengjie Jiang, Xiaozhu Tang, Chao Tang, Zhen Hua, Mengying Ke, Chen Wang, Jiamin Zhao, Shengyao Gao, Artur Jurczyszyn, Siegfried Janz, Meral Beksac, Fenghuang Zhan, Chunyan Gu, Ye Yang
Summary: This study identified that HNRNPA2B1 is upregulated in multiple myeloma (MM) patients and negatively correlated with prognosis, playing a role in regulating cell proliferation by modulating AKT3 expression. This suggests that HNRNPA2B1 may act as a potential therapeutic target for MM through the ILF3-dependent pattern.
JOURNAL OF HEMATOLOGY & ONCOLOGY
(2021)
Article
Cell Biology
Xuxing Shen, Chao Wu, Meng Lei, Qing Yan, Haoyang Zhang, Lina Zhang, Xueyuan Wang, Ye Yang, Jianyong Li, Yongqiang Zhu, Lijuan Chen
Summary: The novel proteasome inhibitor D395 showed high cytotoxicity to MM cells, with lower cardiotoxicity compared to carfilzomib. D395 demonstrated remarkable anti-MM activity and mild cardiotoxicity in both in vitro and in vivo experiments.
CELL DEATH & DISEASE
(2021)
Article
Cell Biology
Mengjie Guo, Pinggang Ding, Zhen Zhu, Lu Fan, Yanyan Zhou, Shu Yang, Ye Yang, Chunyan Gu
Summary: The study showed that elevated RFWD2 expression in MM patients leads to adverse outcomes and drug resistance. RFWD2 is involved in controlling cell cycle, growth and death in MM biology. Targeting RFWD2 may be an effective strategy to inhibit cellular proliferation and overcome drug resistance in MM.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Oncology
Chunyan Gu, Yajun Wang, Lulin Zhang, Li Qiao, Shanliang Sun, Miaomiao Shao, Xiaozhu Tang, Pinggang Ding, Chao Tang, Yuhao Cao, Yanyan Zhou, Mengjie Guo, Rongfang Wei, Nianguang Li, Yibei Xiao, Jinao Duan, Ye Yang
Summary: The study found that AHSA1 expression was increased in multiple myeloma samples, which was significantly associated with disease relapse and poor prognosis. Additionally, AHSA1 played a role in promoting MM cell proliferation and proteasome inhibitor (PI) resistance. Bufalin and KU-177, as selective inhibitors of AHSA1, may be promising targets for treating MM cell proliferation and proteasome inhibitor resistance.
JOURNAL OF EXPERIMENTAL & CLINICAL CANCER RESEARCH
(2022)
Article
Biochemistry & Molecular Biology
Zhen Hua, Rongfang Wei, Mengjie Guo, Zigen Lin, Xichao Yu, Xinying Li, Chunyan Gu, Ye Yang
Summary: This study highlights the role of the YTHDF2/STAT5A/MAP2K2/p-ERK axis in the proliferation of multiple myeloma (MM), and targeting YTHDF2 may be a promising therapeutic strategy.
Article
Pharmacology & Pharmacy
Rongfang Wei, Xing Cui, Jie Min, Zigen Lin, Yanyan Zhou, Mengjie Guo, Xiaojuan An, Hao Liu, Siegfried Janz, Chunyan Gu, Hongbo Wang, Ye Yang
Summary: This study found that NAT10 is significantly upregulated in multiple myeloma (MM) patients and is closely associated with poor prognosis. Enforced expression of NAT10 promotes MM growth, while its knockdown reverses these effects. Further investigation revealed CEP170 as an important downstream target of NAT10. These findings suggest that NAT10 may serve as a promising therapeutic target in MM.
ACTA PHARMACEUTICA SINICA B
(2022)
Article
Oncology
Yuanjiao Zhang, Xichao Yu, Rongze Sun, Jie Min, Xiaozhu Tang, Zigen Lin, Siyuan Xie, Xinying Li, Shengfeng Lu, Zhidan Tian, Chunyan Gu, Lesheng Teng, Ye Yang
Summary: This study found that SFRS8 is upregulated in multiple myeloma and is associated with poor prognosis. SFRS8 can promote the progression of myeloma by regulating the alternative splicing of CACYBP and promote osteoclast differentiation through exosomes. Targeting the SFRS8/CACYBP/beta-catenin axis may be a promising strategy for the diagnosis and treatment of multiple myeloma.
CLINICAL AND TRANSLATIONAL MEDICINE
(2022)
Article
Oncology
Xiaozhu Tang, Zhendong Deng, Pinggang Ding, Wanting Qiang, Yue Lu, Shengyao Gao, Ye Hu, Ye Yang, Juan Du, Chunyan Gu
Summary: The study reveals that circHNRNPU_603aa has potential diagnostic and therapeutic value in multiple myeloma.
JOURNAL OF EXPERIMENTAL & CLINICAL CANCER RESEARCH
(2022)
Review
Pharmacology & Pharmacy
Hongbo Wang, Qinghai Meng, Jinjun Qian, Muxi Li, Chunyan Gu, Ye Yang
Summary: This review provides an overview of different types of RNAs in cancer diagnostics and treatment, discussing their unique characteristics and potential applications. It highlights the increasing use of mRNAs, lncRNAs, circRNAs, and miRNAs as diagnostic markers or therapeutic targets in various cancers. The review also explores the mechanisms by which these RNAs regulate and participate in cancer development, as well as their potential in cancer prediction and treatment.
PHARMACOLOGY & THERAPEUTICS
(2022)
Article
Oncology
Rongfang Wei, Yan Zhu, Yuanjiao Zhang, Wene Zhao, Xichao Yu, Ling Wang, Chunyan Gu, Xiaosong Gu, Ye Yang
Summary: In patients with multiple myeloma (MM), increased expression of AIMP1 is closely associated with unfavorable prognostic outcomes. AIMP1 promotes MM cell proliferation by activating the MAPK signaling pathway and affects bone lesion formation through histone acetylation. Additionally, AIMP1 plays a role in promoting osteoclast differentiation.
CANCER COMMUNICATIONS
(2022)
Review
Oncology
Yiwen Zhang, Lu Lu, Feifeng Song, Xiaozhou Zou, Yujia Liu, Xiaowei Zheng, Jinjun Qian, Chunyan Gu, Ping Huang, Ye Yang
Summary: Non-protein target drugs, especially RNA-based gene therapies, have gained global recognition for treating hereditary diseases. However, there is a lack of miracle drugs for cancer, which remains a challenging condition. As biopharmaceutical research advances, the focus of cancer therapy has shifted towards non-protein targets, particularly RNA therapeutics such as oligonucleotide drugs and mRNA vaccines. This review provides a summary of the clinical research progress in RNA therapeutics, emphasizing the importance of appropriate target selection and optimized delivery vehicles in enhancing the effectiveness of cancer treatment in vivo.
JOURNAL OF EXPERIMENTAL & CLINICAL CANCER RESEARCH
(2023)