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Hematopoietic diseases

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https://www.readbyqxmd.com/read/29785446/digital-droplet-pcr-based-absolute-quantification-of-pre-transplant-npm1-mutation-burden-predicts-relapse-in-acute-myeloid-leukemia-patients
#1
Marius Bill, Juliane Grimm, Madlen Jentzsch, Laura Kloss, Karoline Goldmann, Julia Schulz, Stefanie Beinicke, Janine Häntschel, Michael Cross, Vladan Vucinic, Wolfram Pönisch, Gerhard Behre, Georg-Nikolaus Franke, Thoralf Lange, Dietger Niederwieser, Sebastian Schwind
Allogeneic hematopoietic stem cell transplantation is an established consolidation therapy for patients with acute myeloid leukemia. However, relapse after transplantation remains a major clinical problem resulting in poor prognosis. Thus, detection of measurable ("minimal") residual disease to identify patients at high risk of relapse is essential. A feasible method to determine measurable residual disease may be digital droplet PCR (ddPCR) that allows absolute quantification with high sensitivity and specificity without the necessity of standard curves...
May 22, 2018: Annals of Hematology
https://www.readbyqxmd.com/read/29785172/graft-versus-host-disease-from-basic-pathogenic-principles-to-dna-damage-response-and-cellular-senescence
#2
REVIEW
Adam Kuba, Ludek Raida
Graft versus host disease (GVHD), a severe immunogenic complication of allogeneic hematopoietic stem cell transplantation (HSCT), represents the most frequent cause of transplant-related mortality (TRM). Despite a huge progress in HSCT techniques and posttransplant care, GVHD remains a significant obstacle in successful HSCT outcome. This review presents a complex summary of GVHD pathogenesis with focus on references considering basic biological processes such as DNA damage response and cellular senescence.
2018: Mediators of Inflammation
https://www.readbyqxmd.com/read/29785130/human-herpesvirus-6-pneumonitis-in-a-patient-with-follicular-lymphoma-following-immunochemotherapy-with-rituximab
#3
Saeko Kuwahara-Ota, Yoshiaki Chinen, Yoshimi Mizuno, Tomoko Takimoto-Shimomura, Yayoi Matsumura-Kimoto, Kazuna Tanba, Taku Tsukamoto, Shinsuke Mizutani, Yuji Shimura, Tsutomu Kobayashi, Shigeo Horiike, Junya Kuroda
Primary infection with human herpesvirus-6 (HHV-6) commonly occurs at an early age in children, most often at 3 years of age, and is associated with childhood diseases, such as exanthema subitum, hepatitis, febrile convulsions, or encephalitis. However, the virus occasionally reactivates from its latent state in immunosuppressed adults, especially post-transplant, resulting in serious disseminated, sometimes life-threatening end-organ complications. Herein, we report a case of a 68-year-old man with relapsed follicular lymphoma who developed HHV-6 pneumonitis...
2018: Infection and Drug Resistance
https://www.readbyqxmd.com/read/29782940/defining-osteoblast-and-adipocyte-lineages-in-the-bone-marrow
#4
J L Pierce, D L Begun, J J Westendorf, M E McGee-Lawrence
Bone is a complex endocrine organ that facilitates structural support, protection to vital organs, sites for hematopoiesis, and calcium homeostasis. The bone marrow microenvironment is a heterogeneous niche consisting of multipotent musculoskeletal and hematopoietic progenitors and their derivative terminal cell types. Amongst these progenitors, bone marrow mesenchymal stem/stromal cells (BMSCs) may differentiate into osteogenic, adipogenic, myogenic, and chondrogenic lineages to support musculoskeletal development as well as tissue homeostasis, regeneration and repair during adulthood...
May 18, 2018: Bone
https://www.readbyqxmd.com/read/29782339/transcription-factors-foxo-in-the-regulation-of-homeostatic-hematopoiesis
#5
Vijay Menon, Saghi Ghaffari
PURPOSE OF REVIEW: Work in the past decade has revealed key functions of the evolutionary conserved transcription factors Forkhead box O (FOXO) in the maintenance of homeostatic hematopoiesis. Here the diverse array of FOXO functions in normal and diseased hematopoietic stem and progenitor cells is reviewed and the main findings in the past decade are highlighted. Future work should reveal FOXO-regulated networks whose alterations contribute to hematological disorders. RECENT FINDINGS: Recent studies have identified unanticipated FOXO functions in hematopoiesis including in hematopoietic stem and progenitor cells (HSPC), erythroid cells, and immune cells...
May 17, 2018: Current Opinion in Hematology
https://www.readbyqxmd.com/read/29781813/the-notch1-cd44-axis-drives-pathogenesis-in-a-t-cell-acute-lymphoblastic-leukemia-model
#6
Marina García-Peydró, Patricia Fuentes, Marta Mosquera, María J García-León, Juan Alcain, Antonio Rodríguez, Purificación García de Miguel, Pablo Menéndez, Kees Weijer, Hergen Spits, David T Scadden, Carlos Cuesta-Mateos, Cecilia Muñoz-Calleja, Francisco Sánchez-Madrid, María L Toribio
NOTCH1 is a prevalent signaling pathway in T cell acute lymphoblastic leukemia (T-ALL), but crucial NOTCH1 downstream signals and target genes contributing to T-ALL pathogenesis cannot be retrospectively analyzed in patients and thus remain ill defined. This information is clinically relevant, as initiating lesions that lead to cell transformation and leukemia-initiating cell (LIC) activity are promising therapeutic targets against the major hurdle of T-ALL relapse. Here, we describe the generation in vivo of a human T cell leukemia that recapitulates T-ALL in patients, which arises de novo in immunodeficient mice reconstituted with human hematopoietic progenitors ectopically expressing active NOTCH1...
May 21, 2018: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/29781586/treatment-algorithms-for-systemic-sclerosis-according-to-experts
#7
Andreu Fernández-Codina, Kyle M Walker, Janet E Pope
INTRODUCTION: Treatment for many aspects of systemic sclerosis (SSc) lacks agreement. OBJECTIVES: To generate SSc treatment algorithms endorsed by high percentage of SSc experts. METHODS: Experts from the Scleroderma Clinical Trials Consortium and the Canadian Scleroderma Research group (N=170) were asked whether they agreed with SSc algorithms (from 2012). Two consensus rounds refined agreement; 62 (36%), 54 (32%) and 48 (28%) experts completed surveys...
May 21, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29780847/community-acquired-respiratory-paramyxovirus-infection-after-allogeneic-hematopoietic-cell-transplantation-a-single-center-experience
#8
Yasmin Spahr, Sarah Tschudin-Sutter, Veronika Baettig, Francesca Compagno, Michael Tamm, Jörg Halter, Sabine Gerull, Jakob Passweg, Hans H Hirsch, Nina Khanna
Background: Paramyxoviruses include respiratory syncytial virus (RSV), parainfluenza virus (PIV), and human metapneumovirus (MPV), which may cause significant respiratory tract infectious disease (RTID) and mortality after allogeneic hematopoietic cell transplantation (HCT). However, clinical data regarding frequency and outcome are scarce. Methods: We identified all paramyxovirus RTIDs in allogeneic HCT recipients diagnosed by multiplex polymerase chain reaction between 2010 and 2014...
May 2018: Open Forum Infectious Diseases
https://www.readbyqxmd.com/read/29780814/targeting-chronic-myeloid-leukemia-stem-cells-can-transcriptional-program-be-a-druggable-target-for-cancers
#9
REVIEW
Yosuke Masamoto, Mineo Kurokawa
Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm resulting from acquisition of constitutively active BCR-ABL protein tyrosine kinase in a hematopoietic stem cell (HSC). Though tyrosine kinase inhibitors (TKIs) have changed a fatal disease into manageable disease, most patients cannot discontinue TKI treatment due to persistence of TKI-resistant leukemia stem cells (LSCs). Much effort has been made to find out factors or pathways specifically operating in LSCs to selectively target LSCs, with some promising results at least in preclinical models...
2018: Stem Cell Investigation
https://www.readbyqxmd.com/read/29780388/regulation-of-hematopoietic-cell-development-and-function-through-phosphoinositides
#10
REVIEW
Mila Elich, Karsten Sauer
One of the most paramount receptor-induced signal transduction mechanisms in hematopoietic cells is production of the lipid second messenger phosphatidylinositol(3,4,5)trisphosphate (PIP3 ) by class I phosphoinositide 3 kinases (PI3K). Defective PIP3 signaling impairs almost every aspect of hematopoiesis, including T cell development and function. Limiting PIP3 signaling is particularly important, because excessive PIP3 function in lymphocytes can transform them and cause blood cancers. Here, we review the key functions of PIP3 and related phosphoinositides in hematopoietic cells, with a special focus on those mechanisms dampening PIP3 production, turnover, or function...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29779349/-influence-of-donor-recipient-sex-matching-on-outcomes-of-haploidentical-hematopoietic-stem-cell-transplantation-for-acute-leukemia
#11
W J Yu, Y Wang, L P Xu, X H Zhang, K Y Liu, X J Huang
Objective: To determine the influence of donor-recipient sex matching on outcome of haploidentical hematopoietic stem cell transplantation (haplo-HSCT) for acute leukemia in the setting of T-cell-replete transplants. Methods: The retrospective study is based on 1 160 consecutive patients who received their first haplo-HSCT for acute leukemia between April 2002 and December 2014 at Peking University Institute of Hematology. The patients were divided into the sex-matched group and sex-mismatched group in terms of the recipient and donor sex...
May 14, 2018: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/29778716/the-hematopoietic-stem-cell-niche-what-s-so-special-about-bone
#12
REVIEW
Benjamin J Frisch
Hematopoietic stem cells (HSCs) require a supportive microenvironment to regulate their function and produce sufficient hematopoietic cells over the lifetime of an individual. With the exception of fish, all vertebrates, including mammals, maintain HSCs in a complex niche within the bone marrow. Several bone specific cellular populations have been implicated as components of the HSC niche and are part of a complex network that regulates HSC functions. However, the full extent of interactions within the HSC niche, and the role of individual cell populations remain to be fully elucidated...
May 17, 2018: Bone
https://www.readbyqxmd.com/read/29778715/photopheresis-of-a-less-than-10-kg-child-with-acute-graft-versus-host-disease-accompanied-with-hyperbilirubinemia-a-case-report
#13
REVIEW
Ashraf Malek Mohammadi, Amir Hossein Norooznezhad, Jerard Seghatchian, Mohsen Nikbakht, Farhad Heshmati, Saeed Mohammadi, Kamran Alimoghaddam, Ardeshir Ghavamzadeh
Hematopoietic stem cell transplantation is a curative treatment for many hematologic malignancies with its most important side effect being graft-versus-host disease (GVHD). Herein, we present a 3.5 year-old male with weight of 9.8 kg with acute GVHD (grade IV gastrointestinal and cutaneous) who did not respond to the first line therapies (corticosteroids). Thus, the patient was a candidate for extracorporeal photochemotherapy (ECP). Due to the hyperbilirubinemia, two sessions of ECP every week as well as one session of plasmapheresis 24 h before each ECP session were performed (Spectra™ Optia® apheresis system)...
May 9, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29778713/concluding-commentary-on-current-trends-to-enhance-the-clinical-safety-of-pediatric-transfusion-focusing-on-prevention-of-untoward-complications-of-hsc-transplantation-newer-strategies-for-improving-the-standards-of-safety-quality-of-stem-cells-expansion-for
#14
REVIEW
Irena Sniecinski, Jerard Seghatchian
Clinical practice and related diagnostic, development and research [DDR] strategies in pediatric transfusion and transplantation cover a broad range of multidisciplinary studies, performed by many professionals involved in this most challenging clinical field [1]. This commentary on the current position and future perspectives in pediatric transfusion field is aimed to highlight major unresolved transfusion complications in pediatric patients, namely red blood cell and platelet alloimmunisation, and new ones such as nosocomial infection, thrombosis and multi-organ failure...
May 16, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29777868/comparative-efficacy-and-safety-of-different-antiviral-agents-for-cytomegalovirus-prophylaxis-in-allogeneic-hematopoietic-cell-transplantation-a-systematic-review-and-meta-analysis
#15
Nico Gagelmann, Per Ljungman, Jan Styczynski, Nicolaus Kröger
Over the past 25 years, several randomized controlled trials have investigated the efficacy of different antiviral agents for cytomegalovirus (CMV) prophylaxis in allogeneic hematopoietic-cell transplantation. We performed a systematic literature review, conventional meta-analysis and network meta-analysis using a random-effects model and risk ratios (RR) with corresponding 95% confidence intervals (CI) as effect estimates. Fifteen randomized controlled trials were identified, including seven different antiviral agents: acyclovir, ganciclovir, maribavir, brincidofovir, letermovir, valacyclovir, and vaccine...
May 16, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29777867/antibiotic-exposure-prior-to-respiratory-viral-infection-is-associated-with-progression-to-lower-respiratory-tract-disease-in-allogeneic-hematopoietic-cell-transplant-recipients
#16
Chikara Ogimi, Elizabeth M Krantz, Jonathan L Golob, Alpana Waghmare, Catherine Liu, Wendy M Leisenring, Christopher R Woodard, Sara Marquis, Jane M Kuypers, Keith R Jerome, Steven A Pergam, David N Fredricks, Mohamed L Sorror, Janet A Englund, Michael Boeckh
INTRODUCTION: Recent publications note an association between antibiotic exposure and respiratory viral infections (RVIs). Antibiotics affect microbiota and impair immune response against RVIs in mice, and low microbiome diversity is associated with pulmonary complications including viral lower respiratory tract disease (LRTD) in hematopoietic cell transplantation (HCT) recipients. In this study, we examined whether antibiotic exposure was associated with increased risk of disease progression in RVIs post-transplantation...
May 16, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29777574/factors-influencing-the-umbilical-cord-blood-stem-cell-industry-an-evolving-treatment-landscape
#17
REVIEW
Carla Dessels, Marco Alessandrini, Michael Sean Pepper
Hematopoietic stem cell transplantation (HSCT) is common practice today for life threatening malignant and non-malignant diseases of the blood and immune systems. Umbilical cord blood (UCB) is rich in hematopoietic stem cells (HSCs) and is an attractive alternative to harvesting HSCs from bone marrow or when mobilized into peripheral blood. One of the most appealing attributes of UCB is that it can be banked for future use and hence provides an off-the-shelf solution for patients in urgent need of a transplantation...
May 18, 2018: Stem Cells Translational Medicine
https://www.readbyqxmd.com/read/29777019/cellular-origin-and-functional-relevance-of-collagen-i-production-in-the-kidney
#18
Simone Buchtler, Alexandra Grill, Stefanie Hofmarksrichter, Petra Stöckert, Gabriela Schiechl-Brachner, Manuel Rodriguez Gomez, Sophia Neumayer, Kathrin Schmidbauer, Yvonne Talke, Barbara M Klinkhammer, Peter Boor, Alexander Medvinsky, Kerstin Renner, Hayo Castrop, Matthias Mack
Background Interstitial fibrosis is associated with chronic renal failure. In addition to fibroblasts, bone marrow-derived cells and tubular epithelial cells have the capacity to produce collagen. However, the amount of collagen produced by each of these cell types and the relevance of fibrosis to renal function are unclear. Methods We generated conditional cell type-specific collagen I knockout mice and used (reversible) unilateral ureteral obstruction and adenine-induced nephropathy to study renal fibrosis and function...
May 18, 2018: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29776323/low-frequency-sensorineural-hearing-loss-in-familial-hemophagocytic-lymphohistiocytosis-type-5
#19
Aren Bezdjian, Hanneke Bruijnzeel, Julia Pagel, Sam J Daniel, Hans G X M Thomeer
INTRODUCTION: Familial hemophagocytic lymphohistiocytosis (FHL) is an autosomal recessive disease affecting the cytotoxic pathway. Due to the recent advances in molecular diagnosis, immuno-chemo therapy, and hematopoietic stem cell transplantation treatment, FHL survival rates have drastically increased. CASE PRESENTATION: Herein, we describe a case of FHL type 5 presenting with low-frequency sensorineural hearing loss. Alongside our reported case, 6 additional patients were identified in the literature...
June 2018: Annals of Otology, Rhinology, and Laryngology
https://www.readbyqxmd.com/read/29775636/epithelial-proliferation-in-inflammatory-skin-disease-is-regulated-by-ttc7-in-fibroblasts-and-lymphocytes
#20
Ursina Nüesch, Andrea A Mauracher, Lennart Opitz, Benjamin Volkmer, Katarzyna Michalak-Mićka, Jivko Kamarachev, Tom Hartwig, Ernst Reichmann, Burkhard Becher, Stefano Vavassori, Jana Pachlopnik Schmid
BACKGROUND: Mutations in tetratricopeptide repeat domain 7A (TTC7A) and its mouse orthologue Ttc7 result in a multisystemic disease, mostly affecting epithelial barriers and the immune system. Despite successful hematopoietic stem cell transplantation, ongoing progression of gastrointestinal manifestations can be life-threatening in TTC7A-deficient patients. OBJECTIVE: To identify whether TTC7A mutations dysregulate epithelial cells only, or whether a cell-intrinsic defect in lymphocytes or other cells contributes to disease manifestations...
May 15, 2018: Journal of Allergy and Clinical Immunology
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