Sporadic late-onset nemaline myopathy （SLONM） is a rare， acquired， and treatable myopathy with a subacute or chronic progressive clinical course， characterized by asymmetric muscle atrophy and weakness in the axial and limb muscles， with or without involvement of respiratory and cardiac muscles. The only definitive diagnostic method at present is the identification of rods accumulation in muscle fibers by muscle biopsy pathology. This article provides a review of the clinical manifestations， diagnostic evaluations， muscle pathology， and advances in the treatment of SLONM.
Immunotherapy

Autoimmune encephalitis（AE）is a type of brain inflammation caused by the immune system mistaking autoantigens expressed in the central nervous system for foreign antigens，thereby resulting in abnormal immune response that affects brain parenchyma（cortex or deep gray matter and white matter）and may involve the meninges and spinal cord. This disease is not an infectious inflammatory disease caused by pathogen invasion and is fundamentally different from purulent encephalitis and viral encephalitis，and it can occur in different populations such as children，adolescents，and adults. A recent epidemiological study from the United States shows that there might be a higher prevalence rate of AE，and the prevalence rate of AE was 13.7/100 000 in this population-based study，with no significant difference from the prevalence rate of infectious encephalitis. Since the discovery of anti-N-methyl-D-aspartate（NMDA）receptor antibodies，many patients who experience mental symptoms with rapid progression，abnormal behavior，seizures，or unexplained coma have been diagnosed with AE. The onset of symptoms is usually unclear，which might be similar to mental illness，but then the disease often progresses rapidly and causes damage to brain parenchyma and even loss of consciousness and coma，which usually requires intensive care. Therefore，a comprehensive and systematic understanding of AE is of great significance for clinicians to achieve early identification，diagnosis，and treatment and help patients obtain a good prognosis. This article aims to provide a comprehensive overview of existing research findings of AE in terms of its past，present，and future development and from cognitive limitations to the leap towards precision treatment，in order to provide assistance for diagnosis and treatment among clinicians.
Immunotherapy

BACKGROUND

Intraoperative transfusion for gynecologic surgery, when appropriately used, can improve patient outcomes. However, when utilized incorrectly, blood transfusion can worsen patient outcomes and increase patient cost. This study aimed to evaluate the blood transfusion practices of tertiary hospitals in the Philippines.

The study utilized a cross-sectional design wherein prospective data were gathered through multiple sources across seven tertiary-level hospitals. Women admitted to undergo gynecologic surgery were recruited based on a set of criteria. A chart review was conducted, and blood utilization indices were calculated. Outcomes were compared between public versus private facilities and transfused versus nontransfused patients.

Among 514 patients, 79.7% underwent cross-matching and 75.1% received transfusions. Adverse events were rare, with no transfusion-related deaths. The overall crossmatch-to-transfusion ratio (C/T ratio) was 2.8, exceeding the 2.5 optimal benchmark; all public hospitals recorded a C/T ratio >2.5, whereas private centers had more efficient usage. Six hospitals met acceptable benchmarks for transfusion probability and transfusion index. Open abdominal procedures, particularly hysterectomy, accounted for the most blood used. Transfused patients had longer operative times, greater blood loss, lower preoperative hemoglobin, and more frequently involved resident physicians in training. Public hospitals recorded higher cross-match and transfusion rates, greater resident physician participation, and broader use of general anesthesia.

Results of the study highlight the importance of monitoring blood transfusion parameters to optimize blood utilization. The observed differences between public and private institutions in the country highlight the urgent need for standardized and evidence-based practice to ensure efficient transfusion protocols nationwide.

γδ T cells are a kind of innate immune T cell. They have not attracted sufficient attention because they account for only a small proportion of all immune cells, and many basic factors related to these cells remain unclear. However, in recent years, with the rapid development of tumor immunotherapy, γδ T cells have attracted increasing attention because of their ability to exert cytotoxic effects on most tumor cells without major histocompatibility complex (MHC) restriction. An increasing number of basic studies have focused on the development, antigen recognition, activation, and antitumor immune response of γδ T cells. Additionally, γδ T cell-based immunotherapeutic strategies are being developed, and the number of clinical trials investigating such strategies is increasing. This review mainly summarizes the progress of basic research and the clinical application of γδ T cells in tumor immunotherapy to provide a theoretical basis for further the development of γδ T cell-based strategies in the future.
Humans ; Receptors, Antigen, T-Cell, gamma-delta ; Immunotherapy, Adoptive ; T-Lymphocytes ; Immunotherapy ; Neoplasms/therapy*

Humans ; Nephrotic Syndrome ; Hematopoietic Stem Cell Transplantation ; Tissue Donors ; Transplantation Conditioning ; Graft vs Host Disease

Immunogenic Cell Death ; Prognosis ; Immunotherapy ; Neoplasms

OBJECTIVE: To summarize the progress of the roles and mechanisms of various types of stem cell-based treatments and their combination therapies in both animal studies and clinical trials of lymphedema. METHODS: The literature on stem cell-based treatments for lymphedema in recent years at home and abroad was extensively reviewed, and the animal studies and clinical trials on different types of stem cells for lymphedema were summarized. RESULTS: Various types of stem cells have shown certain effects in animal studies and clinical trials on the treatment of lymphedema, mainly through local differentiation into lymphoid endothelial cells and paracrine cytokines with different functions. Current research focuses on two cell types, adipose derived stem cells and bone marrow mesenchymal stem cells, both of which have their own advantages and disadvantages, mainly reflected in the therapeutic effect of stem cells, the difficulty of obtaining stem cells and the content in vivo. In addition, stem cells can also play a synergistic role in combination with other treatments, such as conservative treatment, surgical intervention, cytokines, biological scaffolds, and so on. However, it is still limited to the basic research stage, and only a small number of studies have completed clinical trials. CONCLUSION Stem cells have great transformation potential in the treatment of lymphedema, but there is no unified standard in the selection of cell types, the amount of transplanted cells, and the timing of transplantation.
Animals ; Endothelial Cells ; Lymphedema/therapy* ; Stem Cell Transplantation ; Cytokines

Tumor aerobic glycolysis is one of the main features of tumor metabolic reprogramming. This abnormal glycolytic metabolism provides bioenergy and biomaterials for tumor growth and proliferation. It is worth noting that aerobic glycolysis will not only provide biological materials and energy for tumor cells, but also help tumor cells to escape immune surveillance through regulation of immune microenvironment, thereby resisting tumor immunotherapy and promoting tumor progression. Based on the pathogenesis of renal cell carcinoma, this paper describes the characteristics of aerobic glycolysis, the effect of glycolytic metabolism on the immune microenvironment of renal cell carcinoma, the effect of glycolysis inhibitors on the immune microenvironment of renal cell carcinoma, and the prospect of glycolysis inhibitors combined with immune checkpoint inhibitors in the treatment of renal cell carcinoma.
Humans ; Carcinoma, Renal Cell/therapy* ; Immunotherapy ; Glycolysis ; Metabolic Reprogramming ; Kidney Neoplasms/therapy* ; Tumor Microenvironment

Hepatocellular carcinoma (HCC) is well characterized as a heterogeneous disease. Its late-stage diagnosis and chemotherapy resistance make it one of the refractory tumors in China. Natural killer (NK) cells play a significant role in immune surveillance. However, NK cells become dysfunctional in the progression of HCC, leading to tumor immune escape. This article reviews the recent progress on different strategies of NK cell-based immunotherapy in treating HCC, including direct adoptive NK cell transfer, gene engineering in NK cell, NK cell receptor targeting, immunosuppressive microenvironment modification, and tumor toxicity enhancement by cytokines or traditional Chinese medicine. These NK cell-based strategies have shown promising therapeutic potential.
Humans ; Carcinoma, Hepatocellular/therapy* ; Liver Neoplasms/therapy* ; Immunotherapy ; Killer Cells, Natural ; Receptors, Natural Killer Cell ; Tumor Microenvironment

BACKGROUND: The application of programmed cell death 1 (PD-1)/programmed cell death ligand 1 (PD-L1) antibodies has greatly improved the clinical outcomes of lung cancer patients. Here, we retrospectively analyzed the efficacy of PD-1 antibody therapy in locally advanced non-surgical or metastatic lung cancer patients, and preliminarily explored the correlation between peripheral blood biomarkers and clinical responses. METHODS: We conducted a single center study that included 61 IIIA-IV lung cancer patients who received PD-1 antibody treatment from March 2020 to December 2021, and collected the medical record data on PD-1 antibody first-line or second-line treatment. The levels of multiple Th1 and Th2 cytokines in the patient's peripheral blood serum, as well as the phenotype of peripheral blood T cells, were detected and analyzed. RESULTS: All the patients completed at least 2 cycles of PD-1 monoclonal antibody treatment. Among them, 42 patients (68.9%) achieved partial response (PR); 7 patients (11.5%) had stable disease (SD); and 12 patients (19.7%) had progressive disease (PD). The levels of peripheral blood interferon gamma (IFN-γ) (P=0.023), tumor necrosis factor α (TNF-α) (P=0.007) and interleukin 5 (IL-5) (P=0.002) before treatment were higher in patients of the disease control rate (DCR) (PR+SD) group than in the PD group. In addition, the decrease in absolute peripheral blood lymphocyte count after PD-1 antibody treatment was associated with disease progression (P=0.023). Moreover, the levels of IL-5 (P=0.0027) and IL-10 (P=0.0208) in the blood serum after immunotherapy were significantly increased compared to baseline. CONCLUSIONS Peripheral blood serum IFN-γ, TNF-α and IL-5 in lung cancer patients have certain roles in predicting the clinical efficacy of anti-PD-1 therapy. The decrease in absolute peripheral blood lymphocyte count in lung cancer patients is related to disease progression, but large-scale prospective studies are needed to further elucidate the value of these biomarkers.
Humans ; Lung Neoplasms/metabolism* ; Interleukin-5/therapeutic use* ; Tumor Necrosis Factor-alpha/therapeutic use* ; Retrospective Studies ; Programmed Cell Death 1 Receptor ; Biomarkers ; Immunotherapy ; Disease Progression ; B7-H1 Antigen