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Engraftment syndrome

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https://www.readbyqxmd.com/read/29291352/recurrent-ecsit-mutation-encoding-v140a-triggers-hyperinflammation-and-promotes-hemophagocytic-syndrome-in-extranodal-nk-t-cell-lymphoma
#1
Haijun Wen, Huajuan Ma, Qichun Cai, Suxia Lin, Xinxing Lei, Bin He, Sijin Wu, Zifeng Wang, Yan Gao, Wensheng Liu, Weiping Liu, Qian Tao, Zijie Long, Min Yan, Dali Li, Keith W Kelley, Yongliang Yang, Huiqiang Huang, Quentin Liu
Hemophagocytic syndrome (HPS) is a fatal hyperinflammatory disease with a poorly understood mechanism that occurs most frequently in extranodal natural killer/T cell lymphoma (ENKTL). Through exome sequencing of ENKTL tumor-normal samples, we have identified a hotspot mutation (c.419T>C) in the evolutionarily conserved signaling intermediate in Toll pathway (ECSIT) gene, encoding a V140A variant of ECSIT. ECSIT-V140A activated NF-κB more potently than the wild-type protein owing to its increased affinity for the S100A8 and S100A9 heterodimer, which promotes NADPH oxidase activity...
January 1, 2018: Nature Medicine
https://www.readbyqxmd.com/read/29288821/reduced-intensity-allogeneic-transplant-for-acute-myeloid-leukemia-and-myelodysplastic-syndrome-using-combined-cd34-selected-haploidentical-graft-and-a-single-umbilical-cord-unit-compared-to-matched-unrelated-donor-stem-cells-in-older-adults
#2
Stephanie B Tsai, Joanna Rhodes, Hongtao Liu, Tsiporah Shore, Michael Bishop, Melissa M Cushing, Usama Gergis, Lucy Godley, Justin Kline, Richard A Larson, Sebastian Mayer, Olatoyosi Odenike, Wendy Stock, Amittha Wickrema, Koen van Besien, Andrew S Artz
Haplo/cord transplantation combines an umbilical cord blood (UCB) graft with CD34-selected haploidentical cells and results in rapid hematopoietic recovery followed by durable UCB engraftment. We compared outcomes of transplants in older patients with acute myeloid leukemia (AML) or high-risk myelodysplastic syndromes (MDS) who received either HLA-matched unrelated donor cells (MUD) or haplo/cord grafts. Between 2007 and 2013, 109 adults ages 50 and older underwent similar reduced intensity conditioning (RIC) with fludarabine and melphalan and antibody-mediated T-cell depletion for AML (n=83) or high risk MDS (n=26) followed by either a MUD (n=68) or haplo/cord (n=41) graft...
December 27, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29196075/outcomes-after-allogeneic-transplant-in-patients-with-wiskott-aldrich-syndrome
#3
Alexander Ngwube, I Celine Hanson, Jordan Orange, Nicholas L Rider, Filiz Seeborg, William Shearer, Lenora Noroski, Sarah Nicholas, Lisa Forbes, Kathryn Leung, Ghadir Sasa, Swati Naik, Meenakshi Hegde, Bilal Omer, Nabil Ahmed, Carl Allen, Stephen Gottschalk, Meng-Fen Wu, Hao Liu, Malcolm Brenner, Helen Heslop, Robert Krance, Caridad Martinez
Wiskott-Aldrich syndrome (WAS) is a rare X-linked disorder characterized by a triad of immunodeficiency, eczema and thrombocytopenia. Currently, hematopoietic stem cell transplant (HSCT) is the most reliable curative treatment with excellent results for patients with HLA-matched family or unrelated donors. However, even after fully myeloablative preparative regimens, mixed donor chimerism is a potential concern. We performed a retrospective chart review of twelve children who underwent allogeneic hematopoietic stem cell transplant for WAS to report our experience...
November 28, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29189513/successful-nonmyeloablative-allogeneic-stem-cell-transplant-in-a-child-with-emberger-syndrome-and-gata2-mutation
#4
Neha Rastogi, Roshini S Abraham, Ritu Chadha, Dhwanee Thakkar, Shruti Kohli, Sagar Nivargi, Satya Prakash Yadav
Emberger syndrome with underlying guanine-adenine-thymine-adenine 2 (GATA2) mutation is a rare disorder and very few successful nonmyeloablative allogeneic hematopoietic stem cell transplants (HSCTs) have been reported. We report a case of Emberger syndrome with GATA2 mutation in a 9-year-old girl who presented with congenital sensorineural deafness, warts, lymphedema, and Myelodysplastic syndrome. Her sister had died of a similar illness. She underwent a nonmyeloablative matched related donor peripheral blood HSCT with rabbit antithymoglobulin (5 mg/kg), fludarabine (160 mg/m), cyclophophamide (29 mg/kg), and total body irradiation (2 Gray)...
November 17, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29168144/a-review-of-chronic-granulomatous-disease
#5
REVIEW
Danielle E Arnold, Jennifer R Heimall
Chronic granulomatous disease (CGD) is a primary immunodeficiency caused by defects in any of the five subunits of the NADPH oxidase complex responsible for the respiratory burst in phagocytic leukocytes. Patients with CGD are at increased risk of life-threatening infections with catalase-positive bacteria and fungi and inflammatory complications such as CGD colitis. The implementation of routine antimicrobial prophylaxis and the advent of azole antifungals has considerably improved overall survival. Nevertheless, life expectancy remains decreased compared to the general population...
December 2017: Advances in Therapy
https://www.readbyqxmd.com/read/29150659/ectopic-foxp3-expression-preserves-primitive-features-of-human-hematopoietic-stem-cells-while-impairing-functional-t-cell-differentiation
#6
F R Santoni de Sio, L Passerini, M M Valente, F Russo, L Naldini, M G Roncarolo, R Bacchetta
FOXP3 is the transcription factor ruling regulatory T cell function and maintenance of peripheral immune tolerance, and mutations in its coding gene causes IPEX autoimmune syndrome. FOXP3 is also a cell-cycle inhibitor and onco-suppressor in different cell types. In this work, we investigate the effect of ectopic FOXP3 expression on HSC differentiation and we challenged this approach as a possible HSC-based gene therapy for IPEX. FOXP3-expressing HSC showed reduced proliferation ability and increased maintenance of primitive markers in vitro in both liquid and OP9-ΔL1 co-cultures...
November 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29131150/neurologic-complications-after-allogeneic-hematopoietic-stem-cell-transplantation-risk-factors-and-impact
#7
M R Dowling, S Li, B R Dey, S L McAfee, H R Hock, T R Spitzer, Y-B Chen, K K Ballen
Neurologic complications (NCs) may be a significant source of morbidity and mortality after hematopoietic cell transplantation (HCT). We performed a retrospective study of 263 consecutive patients undergoing allogeneic HCT for hematological malignancies to determine the incidence, risk factors and clinical impact of NCs in the first 5 years after HCT. We determined the incidence of central nervous system (CNS) infection, intracranial hemorrhage, ischemic stroke, metabolic encephalopathy, posterior reversal encephalopathy syndrome, seizure and peripheral neuropathy...
November 13, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/29108345/mri-based-evaluation-of-multiorgan-iron-overload-is-a-predictor-of-adverse-outcomes-in-pediatric-patients-undergoing-allogeneic-hematopoietic-stem-cell-transplantation
#8
Natalia Maximova, Massimo Gregori, Giulia Boz, Roberto Simeone, Davide Zanon, Giulia Schillani, Floriana Zennaro
The medical records of 44 pediatric patients who underwent allogeneic transplantation from 2011 to 2015 were retrospectively reviewed. Magnetic resonance imaging was used to measure iron concentrations in the liver, spleen, pancreas and bone. These patients were divided into two groups, 18 with non-elevated (< 100 μmol/g; Group 1) liver iron concentration before transplantation and 26 with elevated (> 100 μmol/g; Group 2) concentration . We compared transplant-related outcomes in the two groups. Iron overload was a negative prognostic risk factor for sinusoidal obstruction syndrome (OR = 17), osteoporosis (OR = 6...
October 3, 2017: Oncotarget
https://www.readbyqxmd.com/read/29100399/oxidative-stress-induced-jnk-ap-1-signaling-is-a-major-pathway-involved-in-selective-apoptosis-of-myelodysplastic-syndrome-cells-by-withaferin-a
#9
Karine Z Oben, Sara S Alhakeem, Mary K McKenna, Jason A Brandon, Rajeswaran Mani, Sunil K Noothi, Liu Jinpeng, Shailaja Akunuru, Sanjit K Dhar, Inder P Singh, Ying Liang, Chi Wang, Ahmed Abdel-Latif, Harold F Stills, Daret K St Clair, Hartmut Geiger, Natarajan Muthusamy, Kaoru Tohyama, Ramesh C Gupta, Subbarao Bondada
Myelodysplastic syndromes (MDS) are a diverse group of malignant clonal hematopoietic stem cell disorders characterized by ineffective hematopoiesis, dysplastic cell morphology in one or more hematopoietic lineages, and a risk of progression to acute myeloid leukemia (AML). Approximately 50% of MDS patients respond to current FDA-approved drug therapies but a majority of responders relapse within 2-3 years. There is therefore a compelling need to identify potential new therapies for MDS treatment. We utilized the MDS-L cell line to investigate the anticancer potential and mechanisms of action of a plant-derived compound, Withaferin A (WFA), in MDS...
September 29, 2017: Oncotarget
https://www.readbyqxmd.com/read/29098296/manipulation-of-panx1-activity-increases-the-engraftment-of-transplanted-lacrimal-gland-epithelial-progenitor-cells
#10
Liana V Basova, Xin Tang, Takeshi Umasume, Anastasia Gromova, Tatiana Zyrianova, Taisia Shmushkovich, Alexey Wolfson, Dillon Hawley, Driss Zoukhri, Valery I Shestopalov, Helen P Makarenkova
Purpose: Sjögren's syndrome is a systemic chronic autoimmune inflammatory disease that primarily targets the salivary and lacrimal glands (LGs). Currently there is no cure; therefore, cell-based regenerative therapy may be a viable option. LG inflammation is facilitated by extracellular ATP and mediated by the Pannexin-1 (Panx1) membrane channel glycoprotein. We propose that suppression of inflammation through manipulation of Panx1 activity can stimulate epithelial cell progenitor (EPCP) engraftment...
November 1, 2017: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/29089623/enzalutamide-inhibits-testosterone-induced-growth-of-human-prostate-cancer-xenografts-in-zebrafish-and-can-induce-bradycardia
#11
Nicole Melong, Shelby Steele, Morgan MacDonald, Alice Holly, Colin C Collins, Amina Zoubeidi, Jason N Berman, Graham Dellaire
The zebrafish has become a popular human tumour xenograft model, particularly for solid tumours including prostate cancer (PCa). To date PCa xenotransplantation studies in zebrafish have not been performed in the presence of testosterone, even when employing androgen-dependent cell models, such as the LNCaP cell line. Thus, with the goal of more faithfully modelling the hormonal milieu in which PCa develops in humans, we sought to determine the effects of exogenous testosterone on the growth of LNCaP, or androgen-independent C4-2 cells xenografted into zebrafish embryos...
October 31, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29084206/myeloablative-conditioning-regimens-with-combined-of-haploidentical-and-cord-blood-transplantation-for-myelodysplastic-syndrome-patients
#12
P Ke, X-B Bao, X-H Hu, J Zhuang, X-J Wu, Y-J Liu, X-F He, D-P Wu, S-L Xue, X Ma
The purpose of this study was to evaluate the strategy of haploidentical (HID) stem cell combined with a small doses of umbilical cord blood (UCB) from a third-party donor transplantation (haplo-cord transplant) for treatment of myelodysplastic syndromes (MDS), by comparing with identical-sibling donor (ISD) transplantation. Eighty-five patients were included between January 2012 and December 2015, with a median 40 years old. Forty-eight patients received haplo-cord transplant and 37 patients received ISD transplant...
October 30, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/29057173/pathogenesis-diagnosis-and-therapeutic-strategies-in-whim-syndrome-immunodeficiency
#13
Lauren E Heusinkveld, Erin Yim, Alexander Yang, Ari B Azani, Qian Liu, Ji-Liang Gao, David H McDermott, Philip M Murphy
21 INTRODUCTION: WHIM syndrome is a rare combined primary immunodeficiency disorder caused by autosomal dominant gain-of-function mutations in the chemokine receptor CXCR4. It is the only Mendelian condition known to be caused by mutation of a chemokine or chemokine receptor. As such, it provides a scientific opportunity to understand chemokine-dependent immunoregulation in humans and a medical opportunity to develop mechanism-based treatment and cure strategies. 22 AREAS COVERED: This review covers the clinical features, genetics, immunopathogenesis and clinical management of WHIM syndrome...
2017: Expert Opinion on Orphan Drugs
https://www.readbyqxmd.com/read/28988422/comparison-of-two-cytoreductive-regimens-for-%C3%AE-%C3%AE-t-cell-depleted-haploidentical-hsct-in-pediatric-malignancies-improved-engraftment-and-outcome-with-tbi-based-regimen
#14
Elad Jacoby, Nira Varda-Bloom, Gal Goldstein, Daphna Hutt, Chaim Churi, Helly Vernitsky, Amos Toren, Bella Bielorai
BACKGROUND: Graft manipulation using selective depletion of αβ-T cells provides a source of haploidentical hematopoietic stem cell transplantation (haplo-HSCT) enriched in effector cells. We report our experience implementing this haplo-HSCT for high-risk malignancies in pediatric patients focusing on the conditioning regimen. PROCEDURE: We performed a retrospective study of patients who underwent T-cell receptor αβ-depleted haplo-HSCT for high-risk pediatric malignancies...
October 8, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28918304/haploidentical-transplantation-for-older-patients-with-acute-myeloid-leukemia-and-myelodysplastic-syndrome
#15
Stefan O Ciurea, Mithun V Shah, Rima M Saliba, Sameh Gaballa, Piyanuch Kongtim, Gabriela Rondon, Julianne Chen, Whitney Wallis, Kai Cao, Marina Konopleva, Naval Daver, Jorge Cortes, Farhad Ravandi, Amin Alousi, Sairah Ahmed, Uday Popat, Simrit Parmar, Qaiser Bashir, Oran Betul, Chitra Hosing, Elizabeth J Shpall, Katayoun Rezvani, Issa F Khouri, Partow Kebriaei, Richard E Champlin
Allogeneic stem cell transplantation with HLA-matched donors is increasingly used for older patients with acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS). It remains unclear if haploidentical stem cell transplantation (haploSCT) is a suitable option for older patients with this disease. We analyzed 43 patients with AML/MDS (median age, 61 years) who underwent a haploSCT at our institution. All patients received a fludarabine-melphalan-based reduced-intensity conditioning regimen and post-transplant cyclophosphamide-based graft-versus-host disease (GVHD) prophylaxis...
September 14, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28892085/circulating-endothelial-cell-count-a-reliable-marker-of-endothelial-damage-in-patients-undergoing-hematopoietic-stem-cell-transplantation
#16
C Almici, C Skert, B Bruno, A Bianchetti, R Verardi, A Di Palma, A Neva, S Braga, G Piccinelli, G Piovani, M Malagola, S Bernardi, L Giaccone, L Brunello, M Festuccia, K Baeten, D Russo, M Marini
The physio-pathologic interrelationships between endothelium and GvHD have been better elucidated and have led to definition of the entity 'endothelial GvHD' as an essential early phase prior to the clinical presentation of acute GvHD. Using the CellSearch system, we analyzed circulating endothelial cells (CEC) in 90 allogeneic hematopoietic stem cell transplantation (allo-HSCT) patients at the following time-points: T1 (pre-conditioning), T2 (pre-transplant), T3 (engraftment), T4 (onset of GvHD) and T5 (1 week after steroid treatment)...
September 11, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28882193/fragile-x-mental-retardation-protein-regulates-skeletal-muscle-stem-cell-activity-by-regulating-the-stability-of-myf5-mrna
#17
Ryo Fujita, Victoria Zismanov, Jean-Marie Jacob, Solène Jamet, Krum Asiev, Colin Crist
BACKGROUND: Regeneration of adult tissues relies on adult stem cells that are primed to enter a differentiation program, while typically remaining quiescent. In mouse skeletal muscle, these features are reconciled by multiple translational control mechanisms that ensure primed muscle stem cells (MuSCs) are not activated. In quiescent MuSCs, this concept is illustrated by reversible microRNA silencing of Myf5 translation, mediated by microRNA-31 and fragile X mental retardation protein (FMRP)...
September 7, 2017: Skeletal Muscle
https://www.readbyqxmd.com/read/28839454/donor-cell-origin-high-risk-myelodysplastic-syndrome-synchronous-with-an-intracranial-meningioma-like-tumor-8-years-after-allogeneic-hematopoietic-stem-cell-transplantation-for-chronic-lymphocytic-leukemia
#18
G Brás, C Pinho-Vaz, A Campos
Secondary neoplasias are well known consequences of radiotherapy or chemotherapy for a primary cancer. In this report, we describe two rare secondary neoplasias occurring in the same patient: a meningioma-like intracranial tumor and high-risk myelodysplastic syndrome (MDS) of donor-cells origin, both diagnosed simultaneously, 8 years after an allogeneic hematopoietic stem cell transplantation (allo-HSCT) for chronic lymphocytic leukemia (CLL). Due to an engraftment failure during the first allo-HSCT of a matched related donor for CLL treatment, the salvage treatment was a second allo-HSCT...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28836324/hematopoietic-stem-cell-transplantation-in-children-with-griscelli-syndrome-a-single-center-experience
#19
Baris Kuskonmaz, Deniz Ayvaz, Muge Gokce, Tuba Turul Ozgur, Fatma V Okur, Mualla Cetin, Ilhan Tezcan, Duygu Uckan Cetinkaya
GS2 is a rare autosomal recessive disease characterized by hypopigmentation, variable immunodeficiency with HLH. HSCT is the only curative treatment for GS2. We analyzed the outcome of 10 children with GS2 who underwent HSCT at our center between October 1997 and September 2013. The median age of the patients at transplant was 13.5 months (range, 6-58 months). All of the patients developed HLH before HSCT and received HLH 94 or HLH 2004 protocols. Donors were HLA-identical relatives in 8 patients, HLA-mismatched relatives in 2 patients...
November 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28833039/minimally-manipulated-murine-regulatory-t-cells-purified-by-reversible-fab-multimers-are-potent-suppressors-for-adoptive-t-cell-therapy
#20
Fabian Mohr, Julius Clemens Fischer, Marc Nikolaus, Christian Stemberger, Stefan Dreher, Admar Verschoor, Tobias Haas, Hendrik Poeck, Dirk H Busch
The transfer of regulatory T cells, either freshly isolated, or modified, represents a promising therapeutic approach to dampen misdirected immune responses, like autoimmune diseases, chronic inflammatory syndromes and graft versus host disease. Clinical isolation of highly pure regulatory T cell (Treg) populations is still challenging and labeling reagents can influence their viability and functionality, potentially altering the potency of isolated Treg cell products. Here we show that reversible Fab multimer-based Treg purification can prevent conventional antibody label-induced interferences in vitro and in vivo...
August 17, 2017: European Journal of Immunology
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