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https://www.readbyqxmd.com/read/28538663/the-cytokine-flt3-ligand-in-normal-and-malignant-hematopoiesis
#1
REVIEW
Panagiotis Tsapogas, Ciaran James Mooney, Geoffrey Brown, Antonius Rolink
The cytokine Fms-like tyrosine kinase 3 ligand (FL) is an important regulator of hematopoiesis. Its receptor, Flt3, is expressed on myeloid, lymphoid and dendritic cell progenitors and is considered an important growth and differentiation factor for several hematopoietic lineages. Activating mutations of Flt3 are frequently found in acute myeloid leukemia (AML) patients and associated with a poor clinical prognosis. In the present review we provide an overview of our current knowledge on the role of FL in the generation of blood cell lineages...
May 24, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28538512/necrotizing-ulcer-after-bcg-vaccination-in-a-girl-with-leukocyte-adhesion-deficiency-type-1
#2
Hidemitsu Kurosawa, Tomoyuki Mizukami, Hiroyuki Nunoi, Masaya Kato, Yuya Sato, Mayuko Okuya, Keitaro Fukushima, Yoshihiko Katsuyama, Osamu Arisaka
Leukocyte-adhesion deficiency-1 is a recessively inherited disorder associated with recurrent bacterial infections, severe periodontitis, peripheral leukocytosis, and impaired wound healing. We diagnosed moderate-type leukocyte-adhesion deficiency-1 in a 7-year-old girl who developed a necrotizing ulcer after Bacillus Calmette-Guerin vaccination. The patient showed moderate expression of CD18 in neutrophils with a homozygous splice mutation with c.41_c.58+2dup20 of ITGB2 and experienced recurrent severe infections complicated with systemic lupus erythematosus...
May 22, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28538502/successful-reduced-intensity-allogeneic-transplant-with-full-donor-chimerism-and-good-quality-of-life-in-adolescent-patient-with-wiskott-aldrich-syndrome
#3
Salah Ali, Anna Gacsadi, Elizabeth McDougall, Christine Armstrong, Joerg Krueger, Tal Schechter, Muhammad Ali
Wiskott-Aldrich syndrome (WAS) is an X-linked disease characterized by microthrombocytopenia, eczema, immune deficiency, and autoimmune phenomena. Allogeneic hematopoietic stem cell transplantation (HSCT) is the only curative treatment. Myeloablative conditioning is the most common regimen used for HSCT in patients with WAS to avoid the risk of mixed donor chimerism and autoimmunity post-HSCT. There is limited data on the use of reduced intensity conditioning for HSCT in patients with WAS. Here, we report a case with severe phenotype of WAS transplanted successfully with reduced intensity conditioning, which is an acceptable conditioning regimen and can be considered in patients with WAS with significantly impaired organ functions...
May 22, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28538498/ghrelin-protects-the-thymic-epithelium-from-conditioning-regimen-induced-damage-and-promotes-the-restoration-of-cd4-t-cells-in-mice-after-bone-marrow-transplantation
#4
Jingxia Xu, Junyu S Zhu, Xianyu Tian, Qixin Sun, Jianhui Xu, Yuxian Huang, Yingzhi He, Zhiwei Huang, Bingyi Wu
BACKGROUND: The delay in immune reconstitution after hematopoietic stem cell transplantation (HSCT), especially a delay in central immune reconstitution, leads to opportunistic infections and disease relapse after transplantation and affects the long-term outcome of HSCT. This delay is mainly attributable to thymic damage after myeloablative chemotherapy and radiotherapy METHODS: We established a model of allogeneic bone marrow transplantation (BMT) in mice and administered ghrelin (GRL) 7 days before the conditioning regimen or the day after BMT...
May 24, 2017: Transplantation
https://www.readbyqxmd.com/read/28538171/proliferation-drives-aging-related-functional-decline-in-a-subpopulation-of-the-hematopoietic-stem-cell-compartment
#5
Kristina Kirschner, Tamir Chandra, Vladimir Kiselev, David Flores-Santa Cruz, Iain C Macaulay, Hyun Jun Park, Juan Li, David G Kent, Rupa Kumar, Dean C Pask, Tina L Hamilton, Martin Hemberg, Wolf Reik, Anthony R Green
Aging of the hematopoietic stem cell (HSC) compartment is characterized by lineage bias and reduced stem cell function, the molecular basis of which is largely unknown. Using single-cell transcriptomics, we identified a distinct subpopulation of old HSCs carrying a p53 signature indicative of stem cell decline alongside pro-proliferative JAK/STAT signaling. To investigate the relationship between JAK/STAT and p53 signaling, we challenged HSCs with a constitutively active form of JAK2 (V617F) and observed an expansion of the p53-positive subpopulation in old mice...
May 23, 2017: Cell Reports
https://www.readbyqxmd.com/read/28534115/complexity-of-bone-marrow-hematopoietic-stem-cell-niche
#6
REVIEW
Noboru Asada, Shoichiro Takeishi, Paul S Frenette
Hematopoietic stem cells (HSCs) that produce a variety of hematopoietic lineage cells throughout the life reside in specialized microenvironment called "niche" in the bone marrow (BM) where they are tightly regulated. With the recent advances in experimental technologies enabling the selective deletion of molecules, various types of cells in the BM have been proposed to contribute to HSC niche activity. Among these are stromal cells closely associated with the vasculature. In this review, we provide an overview of recent advances in HSC niche research, and focus on the studies describing the functional roles of perivascular cells for HSC maintenance and mobilization...
May 22, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28533818/a-novel-bcr-abl1-fusion-gene-with-genetic-heterogeneity-indicates-a-good-prognosis-in-a-chronic-myeloid-leukemia-case
#7
Fen Zhou, Runming Jin, Yu Hu, Heng Mei
BACKGROUND: Chronic myelogenous leukemia (CML) is a pluripotent hematopoietic stem cell disorder caused by the fusion of the BCR and ABL1 genes. Quantitative RT-PCR (qRT-PCR) is a routinely performed screening technique to identify BCR-ABL1 fusion genes, but a limitation of this method is its inability to recognize novel fusions that have not been previously characterized. Next-generation sequencing (NGS) is an effective and sensitive detection method for the determination of novel BCR-ABL1 fusion genes as well as previously characterized ones...
2017: Molecular Cytogenetics
https://www.readbyqxmd.com/read/28533407/histone-binding-of-dpf2-mediates-its-repressive-role-in-myeloid-differentiation
#8
Ferdinand M Huber, Sarah M Greenblatt, Andrew M Davenport, Concepcion Martinez, Ye Xu, Ly P Vu, Stephen D Nimer, André Hoelz
Double plant homeodomain finger 2 (DPF2) is a highly evolutionarily conserved member of the d4 protein family that is ubiquitously expressed in human tissues and was recently shown to inhibit the myeloid differentiation of hematopoietic stem/progenitor and acute myelogenous leukemia cells. Here, we present the crystal structure of the tandem plant homeodomain finger domain of human DPF2 at 1.6-Å resolution. We show that DPF2 interacts with the acetylated tails of both histones 3 and 4 via bipartite binding pockets on the DPF2 surface...
May 22, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28533309/aryl-hydrocarbon-receptor-inhibition-promotes-hemato-lymphoid-development-from-human-pluripotent-stem-cells
#9
Mathew G Angelos, Paige N Ruh, Beau R Webber, Robert H Blum, Caitlin D Ryan, Laura Bendzick, Seonhui Shim, Ashley M Yingst, Dejene M Tufa, Michael R Verneris, Dan S Kaufman
The aryl hydrocarbon receptor (AHR) plays an important physiological role in hematopoiesis. AHR is highly expressed in hematopoietic stem/progenitor cells (HSPCs) and inhibition of AHR results in a marked expansion of human umbilical cord blood-derived HSPCs following cytokine stimulation. It is unknown whether AHR also contributes earlier in human hematopoietic development. To model hematopoiesis, human embryonic stem cells (hESCs) were allowed to differentiate in defined conditions in the presence of the AHR antagonist StemReginin-1 (SR-1) or AHR agonist 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD)...
May 22, 2017: Blood
https://www.readbyqxmd.com/read/28533197/incomplete-antibodies-may-reduce-abo-cross-match-incompatibility-a-pilot-study
#10
Mehmet Özen, Soner Yılmaz, Tülin Özkan, Yeşim Özer, Aliye Aysel Pekel, Asuman Sunguroğlu, Günhan Gürman, Önder Arslan
OBJECTIVE: Any erythrocyte transfusion among humans having type A or B blood groups is impossible due to antibodies causing fatal transfusion complications. A cross-match test is performed to prevent immune transfusion complications before transfusion. Our hypothesis is that the Fragment antibody (Fab) part of antibody (incomplete antibody) may be used to prevent an immune stimulus related to complete antibody. Therefore, we designed a pilot study to evaluate the effectiveness of these incomplete antibodies with using cross-match tests...
May 23, 2017: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
https://www.readbyqxmd.com/read/28533196/transformation-of-mycosis-fungoides-sezary-syndrome-clinical-characteristics-and-prognosis
#11
Seçil Vural, Bengü Nisa Akay, Ayşenur Botsalı, Erden Atilla, Nehir Parlak, Aylin Okçu Heper, Hatice Şanlı
INTRODUCTION: Transformed mycosis fungoides (T-MF) is a rare variant of mycosis fungoides (MF) with an aggressive course. OBJECTIVES: In this study we aimed to describe characteristics MF/Sezary syndrome (SS) cases with transformation. MATERIALS AND METHODS: Patients diagnosed with transformed MF (T-MF) among MF/SS patients between 2000 and 2014 in a single center are evaluated retrospectively.Demographic, clinical, laboratory, immunophenotype features, response to treatment, survival and histopathologic features were analyzed...
May 23, 2017: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
https://www.readbyqxmd.com/read/28532525/management-of-risks-from-water-and-ice-from-ice-machines-for-the-very-immunocompromised-host-a-process-improvement-project-prompted-by-an-outbreak-of-rapidly-growing-mycobacteria-on-a-pediatric-hematopoietic-stem-cell-transplant-hsct-unit
#12
Amanda Guspiel, Jeremiah Menk, Andrew Streifel, Keith Messinger, John Wagner, Patricia Ferrieri, Susan Kline
BACKGROUND In 2011, pediatric hematopoietic stem cell transplant (HSCT) patients were moved from an older hospital to a new children's hospital. To minimize bacterial growth in the new hospital's water during construction, the plumbing system was flushed and disinfected before occupancy. However, 6 months after occupancy, an increased incidence of rapidly growing mycobacteria (RGM) was detected in clinical cultures. Over 10 months, 15 pediatric HSCT patients were infected, while no pediatric HSCT patients had been infected in the preceding 12 months...
May 23, 2017: Infection Control and Hospital Epidemiology
https://www.readbyqxmd.com/read/28532344/pre-transplantation-thymic-function-is-associated-with-the-risk-of-acute-graft-versus-host-disease-and-cytomegalovirus-viremia-after-allogeneic-hematopoietic-stem-cell-transplantation
#13
Xin Yang, Yuanxin Sun, Sudong Zhang, Hui Yang, Jialin Wei, Yi He, Donglin Yang, Erlie Jiang, Mingzhe Han, Xuemei Qin, Sizhou Feng
OBJECTIVES: To analyze the kinetics of T-cell subsets and thymic function reconstitution after allogeneic hematopoietic stem cell transplantation (AHSCT); to determine whether sjTREC (signal joint TCR rearrangement excision circle) and CD31-positive recent thymic emigrant (CD31 + RTE) are correlated with acute graft versus host disease (aGVHD) or CMV (cytomegalovirus) viremia after AHSCT. METHODS: Forty-nine patients who underwent AHSCT in our institution were prospectively enrolled...
May 22, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28532177/targeting-non-hodgkin-lymphoma-with-blinatumomab
#14
Sheilagh Sanders, Douglas A Stewart
Management of patients with relapsed or refractory non-Hodgkin lymphoma (NHL) remains challenging, and novel effective agents are eagerly awaited. Blinatumomab is a bispecific T-cell engager, targeting CD19. While blinatumomab's primary clinical use has been in B-cell acute lymphoblastic leukemia (B-ALL), there are increasing data for its use in B-lineage lymphomas. Areas covered: The aim of this review is to highlight the clinical data for blinatumomab use in NHL. Herein, the authors provide an overview of blinatumomab, its mechanism of action, its proven efficacy against B-ALL, and its phase I-II data assessing its use in NHL Expert opinion: Blinatumomab has modest activity in phase I-II trials in NHL, and may represent a means of bridging patients with relapsed disease to hematopoietic stem cell transplant...
May 22, 2017: Expert Opinion on Biological Therapy
https://www.readbyqxmd.com/read/28530671/cardiopulmonary-assessment-of-patients-with-systemic-sclerosis-for-hematopoietic-stem-cell-transplantation-recommendations-from-the-european-society-for-blood-and-marrow-transplantation-autoimmune-diseases-working-party-and-collaborating-partners
#15
REVIEW
D Farge, R K Burt, M-C Oliveira, E Mousseaux, M Rovira, Z Marjanovic, J de Vries-Bouwstra, N Del Papa, R Saccardi, S J Shah, D C Lee, C Denton, T Alexander, D G Kiely, J A Snowden
Systemic sclerosis (SSc) is a rare disabling autoimmune disease with a similar mortality to many cancers. Two randomized controlled trials of autologous hematopoietic stem cell transplantation (AHSCT) for SSc have shown significant improvement in organ function, quality of life and long-term survival compared to standard therapy. However, transplant-related mortality (TRM) ranged from 3-10% in patients undergoing HSCT. In SSc, the main cause of non-transplant and TRM is cardiac related. We therefore updated the previously published guidelines for cardiac evaluation, which should be performed in dedicated centers with expertize in HSCT for SSc...
May 22, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28529843/deconstructing-the-complexity-of-tgf%C3%AE-signaling-in-hematopoietic-stem-cells-quiescence-and-beyond
#16
Ashwini Hinge, Marie-Dominique Filippi
The hematopoietic system is highly dynamic and must constantly produce new blood cells every day. Mature blood cells all derive from a pool of rare long-lived hematopoietic stem cells (HSCs) that are mostly quiescent but occasionally divide and self-renew in order to maintain the stem cell pool and continuous replenishment of mature blood cells throughout life. A tight control of HSC self-renewal, commitment to differentiation and maintenance of quiescence states is necessary for lifelong blood supply. Transforming growth factor-β (TGF-β) is a critical regulator hematopoietic cell functions...
December 2016: Current Stem Cell Reports
https://www.readbyqxmd.com/read/28529646/peptide-vaccination-in-the-presence-of-adjuvants-in-patients-after-hematopoietic-stem-cell-transplantation-with-cd4-t-cell-reconstitution-elicits-consistent-cd8-t-cell-responses
#17
Michael Schmitt, Anita Schmitt, Markus Wiesneth, Angela Hückelhoven, Zeguang Wu, Jürgen Kuball, Lei Wang, Peter Schauwecker, Susanne Hofmann, Marlies Götz, Birgit Michels, Birgit Maccari, Patrick Wuchter, Volker Eckstein, Thomas Mertens, Paul Schnitzler, Hartmut Döhner, Anthony D Ho, Donald W Bunjes, Peter Dreger, Hubert Schrezenmeier, Jochen Greiner
Rationale: Patients receiving an allogeneic stem cell graft from cytomegalovirus (CMV) seronegative donors are particularly prone to CMV reactivation with a high risk of disease and mortality. Therefore we developed and manufactured a novel vaccine and initiated a clinical phase I trial with a CMV phosphoprotein 65 (CMVpp65)-derived peptide. Methods: Ten patients after allogeneic stem cell transplantation received four vaccinations at a biweekly interval. All patients were monitored for CMVpp65 antigenemia...
2017: Theranostics
https://www.readbyqxmd.com/read/28529201/bing-neel-syndrome-with-detectable-myd88-l265p-gene-mutation-as-a-late-relapse-following-autologous-hematopoietic-stem-cell-transplantation-for-waldenstr%C3%A3-m-s-macroglobulinemia
#18
Anna J Kopińska, Grzegorz Helbig, Anna Koclęga, Sławomira Kyrcz-Krzemień
No abstract text is available yet for this article.
June 5, 2017: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
https://www.readbyqxmd.com/read/28528959/role-of-astakine1-in-regulating-transglutaminase-activity
#19
Ratchanok Sirikharin, Kingkamon Junkunlo, Kenneth Söderhäll, Irene Söderhäll
Transglutaminase (TGase) has been implicated in maintaining the undifferentiated stage of hematopoietic stem cells (HSC) in the crayfish Pacifastacus leniusculus. TGase activity has been reported to be regulated by astakine1, an essential crayfish cytokine for inducing new hemocyte synthesis in hematopoietic tissue (HPT). Here, the role of astakine1 in TGase activity regulation and clotting protein (CP) cross-linking was characterized. A reduction in TGase activity was observed by the addition of purified astakine1 in vitro for both endogenous crayfish TGase and a commercial purified guinea pig liver TGase...
May 18, 2017: Developmental and Comparative Immunology
https://www.readbyqxmd.com/read/28528711/clinical-impact-of-colonization-with-multidrug-resistant-organisms-on-outcome-after-autologous-stem-cell-transplantation-a-retrospective-single-center-study
#20
Sebastian Scheich, Claudia Reinheimer, Christian Brandt, Thomas A Wichelhaus, Michael Hogardt, Volkhard A J Kempf, Uta Brunnberg, Christian Brandts, Olivier Ballo, Ivana von Metzler, Johanna Kessel, Hubert Serve, Björn Steffen
During the last years, a significant increase in infections caused by multidrug-resistant organisms (MDRO) has been noticed, resulting in an increase of mortality in all fields of healthcare. Hematological patients are particularly affected by MDRO infections due to disease- and therapy-related immunosuppression. To determine the impact of colonization with MDRO on overall survival, we retrospectively analyzed data from patients undergoing autologous hematopoietic stem cell transplantation at our institution...
May 18, 2017: Biology of Blood and Marrow Transplantation
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