severe aplastic anemia
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2022 ◽  
Vol 386 (1) ◽  
pp. 11-23 ◽  
Author(s):  
Régis Peffault de Latour ◽  
Austin Kulasekararaj ◽  
Simona Iacobelli ◽  
Sofie R. Terwel ◽  
Riley Cook ◽  
...  

2022 ◽  
Vol 12 ◽  
Author(s):  
Xing You ◽  
Qiong Yang ◽  
Kai Yan ◽  
Song-Rong Wang ◽  
Rong-Rong Huang ◽  
...  

Severe aplastic anemia (SAA) is an autoimmune disease characterized by immune-mediated destruction of hematopoietic stem and progenitor cells. Autoreactive CD8+ T cells have been reported as the effector cells; however, the mechanisms regulating their cell activation in SAA remain largely unknown. Here, we performed proteomics and metabolomics analyses of plasma and bone marrow supernatant, together with transcriptional analysis of CD8+ T cells from SAA patients and healthy donors, to find key pathways that are involved in pathogenic CD8+ T-cell activation. We identified 21 differential proteins and 50 differential metabolites in SAA patients that were mainly involved in energy metabolism, complement and coagulation cascades, and HIF-1α signaling pathways. Interestingly, we found that these pathways are also enriched in T cells from SAA patients by analyzing available single-cell RNA sequencing data. Moreover, CD8+ T cells from SAA patients contain a highly activated CD38+ subset, which was increased in the bone marrow of SAA patients and a murine model of SAA. This subset presented enriched genes associated with the glycolysis or gluconeogenesis pathway, HIF-1α signaling pathway, and complement associated pathways, all of which were of importance in T-cell activation. In conclusion, our study reveals new pathways that may regulate CD8+ T-cell activation in SAA patients and provides potential therapeutic targets for SAA treatment.


Author(s):  
Vandana Sharma ◽  
Prabin Kumar ◽  
Rajiv Kumar ◽  
Sushmita Chakraborty ◽  
Manju Namdeo ◽  
...  

Abstract Acquired aplastic anemia (aAA) is an autoimmune disease, characterized by infiltration of T lymphocytes in the bone marrow with destruction of hematopoietic stem cells by the effector cells. Interferon gamma (IFN-γ) and perforin are important mediators of cell destruction. In this flow cytometry-based study, we have investigated the percentage of intracellular IFN-γ + and perforin + CD5 + T cells in peripheral blood of newly diagnosed aAA patients before and after immunosuppressive therapy (IST). Patients were categorized as per standard disease severity and response to IST. The median percentage of IFN-γ + and perforin + CD5 + T cells was higher in untreated patients compared to healthy controls. The percentage of these cells was also increased in untreated severe and very severe aplastic anemia when compared with non-severe aplastic anemia patients. In patients before and after IST the median percentage of T cells producing IFN-γ and perforin was elevated in non-responders as compared to partial plus complete responders. The higher percentage of IFN-γ + and perforin + CD5 + T cells may be useful as an early diagnostic marker for aberrant activation of immune system and predict poor response to IST in aAA patients, who will benefit from alternative therapy.


2021 ◽  
pp. 102782
Author(s):  
Shotaro Tabata ◽  
Hiroki Hosoi ◽  
Shogo Murata ◽  
Satomi Takeda ◽  
Toshiki Mushino ◽  
...  

Author(s):  
fangfang Yuan ◽  
MingYue Zhao ◽  
Nan Ma ◽  
Shanggang Zong ◽  
Gangping Li ◽  
...  

Objectives: The purpose of our study was to analyze the co-transplantation efficacy of umbilical cord mesenchymal stem cells (UC-MSCs) and peripheral blood stem cells (PBSCs), which is considered as a novel approach for refractory severe aplastic anemia (RSAA) in children and adolescents. Methods: Thirty-two children and adolescents with RSAA were retrospectively reviewed. According to the source of PBSCs, all patients were divided into two groups (matched sibling donor group and matched unrelated donor group). Engraftment, graft-versus-host disease (GVHD) and overall survival (OS) were analyzed. Results: No adverse events related to UC-MSCs infusion occurred in all patients. The median time for neutrophil engraftment was 13 (10~23) days and 15 (11~28) days for platelet. Grade Ⅰ ~ Ⅱ acute GVHD and moderate chronic GVHD were observed in 21.88% and 12.50% of the cases. No statistically significance was observed between the MSD and MUD group on engraftment, GVHD and complications including infection and hemorrhagic cystitis. The median follow-up time was 38.60 (1.37~140.83) months. To the date of October 31th 2021, 5 died and 27 (84.38%) survived. The 5-year OS rate was not statistically significant between the MSD and MUD group (84.8% ± 10.0% vs 82.4% ± 9.2%, P = 0.674). Conclusions: The application of UC-MSCs in the treatment of RSAA in PBSC transplantation is reliable and safe, which can significantly reduce the incidence of GVHD and severe transplantation-related complications and effectively improve patients’ life quality. Therefore, the method can be used as an active treatment option for patients with RSAA.


2021 ◽  
Author(s):  
Rohini Chakravarthy ◽  
Meghan L. Murphy ◽  
Mary Ann Thompson ◽  
Heather L. McDaniel ◽  
Sara Zarnegar‐Lumley ◽  
...  

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