Pediatric hemophagocytic lymphohistiocytosis

SW Canna, RA Marsh - Blood, The Journal of the American …, 2020 - ashpublications.org
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome describing patients with severe
systemic hyperinflammation. Characteristic features include unremitting fever, cytopenias …

Evolution of our understanding of XIAP deficiency

ACA Mudde, C Booth, RA Marsh - Frontiers in Pediatrics, 2021 - frontiersin.org
X-linked inhibitor of apoptosis (XIAP) deficiency is a rare inborn error of immunity first
described in 2006. XIAP deficiency is characterised by immune dysregulation and a broad …

Immunomodulatory and regenerative effects of MSC-derived extracellular vesicles to treat acute GVHD

S Fujii, Y Miura - Stem Cells, 2022 - academic.oup.com
The development of human mesenchymal stromal/stem cell (MSC)-based therapy has
focused on exploring biological nanoparticles secreted from MSCs. There is emerging …

Quercetin ameliorates XIAP deficiency–associated hyperinflammation

SCC Chiang, E Owsley, N Panchal… - Blood, The Journal …, 2022 - ashpublications.org
XIAP (X-linked inhibitor of apoptosis) deficiency is a rare inborn error of immunity. XIAP
deficiency causes hyperinflammatory disease manifestations due to dysregulated TNF …

Immunopathology and biology-based treatment of steroid-refractory graft-versus-host disease

T Toubai, J Magenau - Blood, The Journal of the American …, 2020 - ashpublications.org
Acute graft-versus-host disease (GVHD) is 1 of the major life-threating complications after
allogeneic cell transplantation. Although steroids remain first-line treatment, roughly one-half …

[HTML][HTML] Challenges and opportunities targeting mechanisms of epithelial injury and recovery in acute intestinal graft-versus-host disease

SA Jansen, EES Nieuwenhuis, AM Hanash… - Mucosal …, 2022 - Elsevier
Despite advances in immunosuppressive prophylaxis and overall supportive care,
gastrointestinal (GI) graft-versus-host disease (GVHD) remains a major, lethal side effect …

Baicalin regulates autophagy to interfere with small intestinal acute graft-versus-host disease

X Sun, M Pisano, L Xu, F Sun, J Xu, W Zheng, X Liu… - Scientific Reports, 2022 - nature.com
Acute graft-versus-host disease (aGVHD) is the main complication of and cause of death
after allogeneic hematopoietic stem cell transplantation. Baicalin can protect the small …

Autophagy differentially regulates tissue tolerance of distinct target organs in graft-versus-host disease models

K Oravecz-Wilson, E Lauder, A Taylor… - The Journal of …, 2024 - Am Soc Clin Investig
Tissue-intrinsic mechanisms that regulate severity of systemic pathogenic immune-mediated
diseases, such as acute graft-versus-host disease (GVHD), remain poorly understood …

Reduced-intensity/reduced-toxicity conditioning approaches are tolerated in XIAP deficiency but patients fare poorly with acute GVHD

DE Arnold, R Nofal, C Wakefield, K Lehmberg… - Journal of Clinical …, 2022 - Springer
X-linked inhibitor of apoptosis (XIAP) deficiency is an inherited primary immunodeficiency
characterized by chronic inflammasome overactivity and associated with hemophagocytic …

How does transfusion-associated graft-versus-host disease compare to hematopoietic cell transplantation-associated graft-versus-host disease?

K Aizawa, D Peltier, E Matsuki, T Toubai - Transfusion and Apheresis …, 2022 - Elsevier
Transfusion-associated graft-versus-host disease (TA-GVHD) is a rare life-threatening
complication of blood transfusion caused by donor T cells that escape rejection by the …