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https://www.readbyqxmd.com/read/27913534/myelodysplastic-and-myeloproliferative-disorders-of-childhood
#1
Henrik Hasle
Myelodysplastic syndrome (MDS) and myeloproliferative disorders are rare in children; they are divided into low-grade MDS (refractory cytopenia of childhood [RCC]), advanced MDS (refractory anemia with excess blasts in transformation), and juvenile myelomonocytic leukemia (JMML), each with different characteristics and management strategies. Underlying genetic predisposition is recognized in an increasing number of patients. Germ line GATA2 mutation is found in 70% of adolescents with MDS and monosomy 7. It is challenging to distinguish RCC from aplastic anemia, inherited bone marrow failure, and reactive conditions...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27910249/the-hla-a-b-and-drb1-polymorphism-in-a-large-dataset-of-south-brazil-bone-marrow-donors-from-rio-grande-do-sul
#2
J A Boquett, J M Nunes, S Buhler, M Z de Oliveira, L F Jobim, M Jobim, N J R Fagundes, L Schüler-Faccini, A Sanchez-Mazas
Human leukocyte antigen (HLA) genes are very informative in population genetics studies and their variability has been widely used to reconstruct the history of geographic and/or demographic expansions of human populations. The characterization of HLA diversity at the population level is also fundamental in clinical studies, particularly for bone marrow transplantation programs. In this study, we investigated the HLA molecular variation in Rio Grande do Sul, South Brazil, in order to identify possible regional differences across this state...
December 2, 2016: HLA
https://www.readbyqxmd.com/read/27894122/b-cell-lymphoma-2-modified-bone-marrow-derived-mesenchymal-stem-cells-transplantation-for-the-treatment-of-diabetes-mellitus-induced-erectile-dysfunction-in-a-rat-model
#3
Xiang Sun, Long-Hua Luo, Liang Feng, Dong-Shui Li, Ke-Zhao Zhong
OBJECTIVE: The study aimed to explore the effects of B cell lymphoma-2 (Bcl-2)-modified bone marrow-derived mesenchymal stem cells (BMSCs) transplantation for the treatment of diabetes mellitus-induced erectile dysfunction (DMED) in a rat model. METHODS: The DMED rat model was successfully established. Thirty-six DMED rats were assigned into the Bcl-2-BMSCs, null-BMSCs, BMSCs and phosphate buffered saline (PBS) groups. Meanwhile, 9 normal rats injected with PBS were taken as the normal control group...
November 29, 2016: Urologia Internationalis
https://www.readbyqxmd.com/read/27892944/veno-occlusive-disease-sinusoidal-obstruction-syndrome-after-haematopoietic-stem-cell-transplantation-middle-east-north-africa-regional-consensus-on-prevention-diagnosis-and-management
#4
A H Al Jefri, H Abujazar, A Al-Ahmari, A Al Rawas, Z Al Zahrani, A Alhejazi, M A Bekadja, A Ibrahim, M Lahoucine, S Ousia, A Bazarbachi
Veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) of the liver is a serious, early complication of haematopoietic stem cell transplantation (HSCT), severe and very severe forms of which are associated with a high mortality rate. A wide variety of patient, disease and treatment-related risk factors for VOD/SOS have been identified. Several bodies have published recommendations for the diagnosis, prevention and management of VOD/SOS following HSCT. A group of regional experts have developed a consensus statement on the diagnosis, prevention and management of VOD/SOS in the Middle East and North Africa region to help in the management of HSCT patients in the region...
November 28, 2016: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/27888632/ventx-induces-expansion-of-primitive-erythroid-cells-and-contributes-to-the-development-of-acute-myeloid-leukemia-in-mice
#5
Eva Gentner, Naidu M Vegi, Medhanie A Mulaw, Tamoghna Mandal, Shiva Bamezai, Rainer Claus, Alpaslan Tasdogan, Leticia Quintanilla-Martinez, Alexander Grunenberg, Konstanze Döhner, Hartmut Döhner, Lars Bullinger, Torsten Haferlach, Christian Buske, Vijay P S Rawat, Michaela Feuring-Buske
Homeobox genes are key regulators in normal and malignant hematopoiesis. The human Vent-like homeobox gene VENTX, a putative homolog of the Xenopus laevis Xvent-2 gene, was shown to be highly expressed in normal myeloid cells and in patients with acute myeloid leukemia. We now demonstrate that constitutive expression of VENTX suppresses expression of genes responsible for terminal erythroid differentiation in normal CD34+ stem and progenitor cells. Transplantation of bone marrow progenitor cells retrovirally engineered to express VENTX caused massive expansion of primitive erythroid cells and partly acute erythroleukemia in transplanted mice...
November 24, 2016: Oncotarget
https://www.readbyqxmd.com/read/27885959/bone-marrow-stromal-cell-sheets-may-promote-axonal-regeneration-and-functional-recovery-with-suppression-of-glial-scar-formation-after-spinal-cord-transection-injury-in-rats
#6
Akinori Okuda, Noriko Horii-Hayashi, Takayo Sasagawa, Takamasa Shimizu, Hideki Shigematsu, Eiichiro Iwata, Yasuhiko Morimoto, Keisuke Masuda, Munehisa Koizumi, Manabu Akahane, Mayumi Nishi, Yasuhito Tanaka
OBJECTIVE Transplantation of bone marrow stromal cells (BMSCs) is a theoretical potential as a therapeutic strategy in the treatment of spinal cord injury (SCI). Although a scaffold is sometimes used for retaining transplanted cells in damaged tissue, it is also known to induce redundant immunoreactions during the degradation processes. In this study, the authors prepared cell sheets made of BMSCs, which are transplantable without a scaffold, and investigated their effects on axonal regeneration, glial scar formation, and functional recovery in a completely transected SCI model in rats...
November 25, 2016: Journal of Neurosurgery. Spine
https://www.readbyqxmd.com/read/27879593/triptolide-reduces-the-required-dose-of-tacrolimus-by-attenuating-inflammation-enhancing-immunosuppression-and-increasing-donor-chimerism-in-a-heterotopic-hindlimb-transplantation-model
#7
Chuan Gu, Fei Liu, Xusong Luo, Xianyu Zhou, Jun Yang, L Scott Levin
BACKGROUND: Induction of tolerance and minimizing the toxicity of immunosuppression are two fundamental goals in vascularized composite allotransplantation. Accumulating data indicate that triptolide is an agent that may have the capacity to suppress inflammation and immunologic rejection. METHODS: A heterotopic hindlimb allotransplantation model from Brown Norway to Lewis rats was established and treated with different doses of tacrolimus combined with or without triptolide...
December 2016: Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/27872734/allogeneic-hematopoietic-stem-cell-transplantation-for-adult-patients-with-fanconi-anemia
#8
Hosein Kamranzadeh Fumani, Mohammad Zokaasadi, Amir Kasaeian, Kamran Alimoghaddam, Asadollah Mousavi, Babak Bahar, Mohammad Vaezi, Ardeshir Ghavamzadeh
BACKGROUND AND OBJECTIVES: Fanconi anemia (FA) is a rare genetic disorder caused by an impaired DNA repair mechanism which leads to an increased tendency toward malignancies and progressive bone marrow failure. The only curative management available for hematologic abnormalities in FA patients is hematopoietic stem cell transplantation (HSCT). This study aimed to report the results of HSCT in adult or adolescent FA patients. PATIENTS AND METHODS: Twenty FA patients with ages of 16 or more who underwent HSCT between 2002 and 2015 enrolled in this study...
2016: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/27869806/preliminary-efficacy-of-a-brief-family-intervention-to-prevent-declining-quality-of-life-secondary-to-parental-bone-marrow-transplantation
#9
B L Fife, D M Von Ah, M L Spath, M T Weaver, Z Yang, T Stump, S Farag
The primary purpose of this research was to develop and evaluate the efficacy and feasibility of a brief, cost-effective family-focused intervention to promote adaptive coping and quality of life throughout a parent's bone marrow transplantation (BMT). Targeted outcomes were cohesion, decreased use of avoidance coping, open communication and effective management of emotional distress. Participants included an intervention group of 31 families and 29 families in a control group who received usual care. Each family included the BMT recipient, a partner/caregiver and children 10-18 years old...
November 21, 2016: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/27869551/critical-limb-ischaemia-and-the-response-to-bone-marrow-derived-cell-therapy-according-to-tcpo2-measurement
#10
Mario Maufus, Marie Antoinette Sevestre-Pietri, Carmine Sessa, Bernard Pignon, Harald Egelhofer, Stephane Dupas, Philippe Nguyen, Celine Genty, Gilles Pernod
BACKGROUND: Cell therapy is an emerging potential biotherapy for critical limb ischaemia (CLI) patients who are not eligible for revascularization. However, the findings on this technique's efficacy are inconsistent. Trials investigating this topic focused on the more severe CLI patients who were often beyond any therapy. Therefore, identifying those who may truly benefit from cell transplantation is now warranted. To this end, we studied the prognostic value of tcPO2 for major amputation after 1 year in patients treated with bone marrow-derived cells...
November 21, 2016: VASA. Zeitschrift Für Gefässkrankheiten
https://www.readbyqxmd.com/read/27865438/evaluation-of-bone-marrow-derived-mesenchymal-stem-cells-for-full-thickness-wound-healing-in-comparison-to-tissue-engineered-chitosan-scaffold-in-rabbit
#11
Mohammad Hossein Rajabian, Gholam Hossein Ghorabi, Bita Geramizadeh, Safoura Sameni, Maryam Ayatollahi
BACKGROUND: Chronic wounds present a major challenge in modern medicine. Even under optimal conditions, the healing process may lead to scarring and fibrosis. The ability of mesenchymal stem cells (MSCs) to differentiate into other cell types makes these cells an attractive therapeutic tool for cell transplantation. Both tissue-engineered construct and MSC therapy are among the current wound healing procedures and potential care. Chitosan has been widely applied in tissue engineering because of its biocompatibility and biodegradability...
November 9, 2016: Tissue & Cell
https://www.readbyqxmd.com/read/27862942/a-novel-xenogeneic-graft-versus-host-disease-model-for-investigating-the-pathological-role-of-human-cd4-or-cd8-t-cells-using-immunodeficient-nog-mice
#12
Ryoji Ito, Ikumi Katano, Kenji Kawai, Mika Yagoto, Takeshi Takahashi, Yuyo Ka, Tomoyuki Ogura, Riichi Takahashi, Mamoru Ito
Graft-versus-host disease (GVHD) is a major complication of allogenic bone marrow transplantation and involves the infiltration of donor CD4+ and/or CD8+ T cells into various organs of the recipient. The pathological role of human CD4+ and CD8+ T cells in GVHD remains controversial. In this study, we established two novel xenogeneic (xeno)-GVHD models. Human CD4+ or CD8+ T cells were purified from peripheral blood and were transplanted into immunodeficient NOD/Shi-scid IL2rg(null) (NOG) mice. Human CD8+ T cells did not induce major GVHD symptoms in conventional NOG mice...
November 15, 2016: American Journal of Transplantation
https://www.readbyqxmd.com/read/27859595/recipient-bone-marrow-derived-stromal-cells-prolong-graft-survival-in-a-rat-hind-limb-allotransplantation-model
#13
Ryosuke Ikeguchi, Ryosuke Kakinoki, Souichi Ohta, Hiroki Oda, Hirofumi Yurie, Yukitoshi Kaizawa, Hiroto Mitsui, Tomoki Aoyama, Junya Toguchida, Shuichi Matsuda
BACKGROUND: Recent studies have indicated that bone marrow-derived stromal cells (BMSCs) have immunomodulatory properties that suppress the T cell responses that cause graft rejection. The purpose of this study is to evaluate the effect of recipient BMSCs intravenous infusion for immunomodulation in a rat vascularized composite allotransplantation model. METHODS: A total of nine Wistar (WIS) rats and thirty Lewis (LEW) rats were used. BMSCs were harvested from three LEW rats...
November 17, 2016: Microsurgery
https://www.readbyqxmd.com/read/27856369/outcomes-of-maintenance-therapy-with-bortezomib-post-autologous-stem-cell-transplantation-for-patients-with-multiple-myeloma
#14
Dharshan Sivaraj, Michael M Green, Zhiguo Li, Anthony D Sung, Stefanie Sarantopoulos, Yubin Kang, Gwynn D Long, Mitchell E Horwitz, Richard D Lopez, Keith M Sullivan, David A Rizzieri, Nelson J Chao, Cristina Gasparetto
Comprehensive recommendations for maintenance therapy post-autologous stem cell transplantation (ASCT) for patients with multiple myeloma (MM) have yet to be defined. Bortezomib has been utilized as maintenance therapy after ASCT, but data attesting to the safety and efficacy of this agent compared to lenalidomide in the post-ASCT setting are limited. Therefore we retrospectively analyzed the outcomes of 102 MM patients who received maintenance therapy with bortezomib post-ASCT at Duke University's Adult Bone Marrow Transplant Clinic (ABMT) between 2005 and 2015...
November 14, 2016: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/27853082/early-improvement-in-marrow-fibrosis-following-haploidentical-stem-cell-transplantation-for-a-patient-with-myelodysplastic-syndrome-with-bone-marrow-fibrosis
#15
Shuichiro Takahashi, Riko Tsumanuma, Keiko Aizawa, Mitsumasa Osakabe, Kunihiko Maeda, Ejiro Omoto
The prognosis for myelodysplastic syndrome with bone marrow fibrosis (MDS-F) is worse than the prognosis of MDS without fibrosis. Hematopoietic stem cell transplantation (HSCT) is the only curative therapy; however, the indications and the procedures involved in HSCT remain unclear. We herein describe a 69-year-old Japanese man with MDS-F who received haploidentical HSCT and post-transplantation cyclophosphamide. Although the first HSCT resulted in secondary graft failure, the second HSCT using PTCy led to successful engraftment after early improvement in fibrosis...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27837103/dendritic-cell-klf2-expression-regulates-t-cell-activation-and-proatherogenic-immune-responses
#16
Noah Alberts-Grill, Daniel Engelbertsen, Dexiu Bu, Amanda Foks, Nir Grabie, Jan M Herter, Felicia Kuperwaser, Tao Chen, Gina Destefano, Petr Jarolim, Andrew H Lichtman
Dendritic cells (DCs) have been implicated as important regulators of innate and adaptive inflammation in many diseases, including atherosclerosis. However, the molecular mechanisms by which DCs mitigate or promote inflammatory pathogenesis are only partially understood. Previous studies have shown an important anti-inflammatory role for the transcription factor Krüppel-like factor 2 (KLF2) in regulating activation of various cell types that participate in atherosclerotic lesion development, including endothelial cells, macrophages, and T cells...
December 15, 2016: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/27829385/complete-response-of-extramedullary-relapse-in-breast-of-acute-t-lymphoblastic-leukemia-after-bone-marrow-transplantation-to-chemoradiotherapy-a-case-report-and-literature-review
#17
Bailong Liu, Bin Liu, Xu Wang, Liang Guo, Xiaoliang Liu, Wei Han, Lihua Dong, Min Liu
BACKGROUND: Relapse of acute lymphoblastic leukemia (ALL) occurring in the breast after allografting is extremely rare, with only 22 reported cases in the literature thus far. Further, the lack of a systemic analysis provides little information about this entity. We present a case of isolated extramedullary relapse from acute T lymphoblastic leukemia (ATLL) after allogeneic hematopoietic stem cell transplantation (HSCT). CASE PRESENTATION: A 32-year-old Chinese woman diagnosed with ATLL with myeloid antigen expression received HSCT from her human leukocyte antigen (HLA)-matched sister and presented with two lesions in her right breast 6 months later...
November 9, 2016: BMC Cancer
https://www.readbyqxmd.com/read/27824136/lrba-is-essential-for-allogeneic-responses-in-bone-marrow-transplantation
#18
Mi Young Park, Raki Sudan, Neetu Srivastava, Sudha Neelam, Christie Youngs, Jia-Wang Wang, Robert W Engelman, William G Kerr
The PH-BEACH-WD40 (PBW) protein family members play a role in coordinating receptor signaling and intracellular vesicle trafficking. LPS-Responsive-Beige-like Anchor (LRBA) is a PBW protein whose immune function remains elusive. Here we show that LRBA-null mice are viable, but exhibit compromised rejection of allogeneic, xenogeneic and missing self bone-marrow grafts. Further, we demonstrate that LRBA-null Natural Killer (NK) cells exhibit impaired signaling by the key NK activating receptors, NKp46 and NKG2D...
November 8, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27819671/protease-activated-receptor-1-inhibits-proliferation-but-enhances-leukemia-stem-cell-activity-in-acute-myeloid-leukemia
#19
S Goyama, M Shrestha, J Schibler, L Rosenfeldt, W Miller, E O'Brien, B Mizukawa, T Kitamura, J S Palumbo, J C Mulloy
Eradication of leukemia stem cells (LSCs) is the ultimate goal of treating acute myeloid leukemia (AML). We recently showed that the combined loss of Runx1/Cbfb inhibited the development of MLL-AF9-induced AML. However, c-Kit(+)/Gr-1(-) cells remained viable in Runx1/Cbfb-deleted cells, indicating that suppressing RUNX activity may not eradicate the most immature LSCs. In this study, we found upregulation of several hemostasis-related genes, including the thrombin-activatable receptor PAR-1 (protease-activated receptor-1), in Runx1/Cbfb-deleted MLL-AF9 cells...
November 7, 2016: Oncogene
https://www.readbyqxmd.com/read/27817074/potential-role-of-wnt-%C3%AE-catenin-signaling-in-blastic-transformation-of-chronic-myeloid-leukemia-cross-talk-between-%C3%AE-catenin-and-bcr-abl
#20
Jing Hu, Min Feng, Zhang-Ling Liu, Yi Liu, Zheng-Lan Huang, Hui Li, Wen-Li Feng
Chronic myeloid leukemia (CML) results from malignant transformation of hematopoietic stem cells induced by the BCR-ABL oncogene. Transformation from chronic to blastic phase is the lethal step in CML. Leukemic stem cells (LSCs) are the basic reason for blastic transformation. It has been shown that Wnt/β-catenin signaling contributes to the self-renewal capacity and proliferation of LSCs in CML. However, the role of Wnt/β-catenin signaling in blastic transformation of CML is still obscure. Here, we explored the relationship between BCR-ABL and β-catenin signaling in vitro and in vivo...
November 5, 2016: Tumour Biology: the Journal of the International Society for Oncodevelopmental Biology and Medicine
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