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Bone marrow dysfunction

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https://www.readbyqxmd.com/read/29344583/heterozygous-rtel1-variants-in-bone-marrow-failure-and-myeloid-neoplasms
#1
Judith C W Marsh, Fernanda Gutierrez-Rodrigues, James Cooper, Jie Jiang, Shreyans Gandhi, Sachiko Kajigaya, Xingmin Feng, Maria Del Pilar F Ibanez, Flávia S Donaires, João P Lopes da Silva, Zejuan Li, Soma Das, Maria Ibanez, Alexander E Smith, Nicholas Lea, Steven Best, Robin Ireland, Austin G Kulasekararaj, Donal P McLornan, Anthony Pagliuca, Isabelle Callebaut, Neal S Young, Rodrigo T Calado, Danielle M Townsley, Ghulam J Mufti
Biallelic germline mutations in RTEL1 (regulator of telomere elongation helicase 1) result in pathologic telomere erosion and cause dyskeratosis congenita. However, the role of RTEL1 mutations in other bone marrow failure (BMF) syndromes and myeloid neoplasms, and the contribution of monoallelic RTEL1 mutations to disease development are not well defined. We screened 516 patients for germline mutations in telomere-associated genes by next-generation sequencing in 2 independent cohorts; one constituting unselected patients with idiopathic BMF, unexplained cytopenia, or myeloid neoplasms (n = 457) and a second cohort comprising selected patients on the basis of the suspicion of constitutional/familial BMF (n = 59)...
January 9, 2018: Blood Advances
https://www.readbyqxmd.com/read/29339450/cardiac-macrophages-promote-diastolic-dysfunction
#2
Maarten Hulsmans, Hendrik B Sager, Jason D Roh, María Valero-Muñoz, Nicholas E Houstis, Yoshiko Iwamoto, Yuan Sun, Richard M Wilson, Gregory Wojtkiewicz, Benoit Tricot, Michael T Osborne, Judy Hung, Claudio Vinegoni, Kamila Naxerova, David E Sosnovik, Michael R Zile, Amy D Bradshaw, Ronglih Liao, Ahmed Tawakol, Ralph Weissleder, Anthony Rosenzweig, Filip K Swirski, Flora Sam, Matthias Nahrendorf
Macrophages populate the healthy myocardium and, depending on their phenotype, may contribute to tissue homeostasis or disease. Their origin and role in diastolic dysfunction, a hallmark of cardiac aging and heart failure with preserved ejection fraction, remain unclear. Here we show that cardiac macrophages expand in humans and mice with diastolic dysfunction, which in mice was induced by either hypertension or advanced age. A higher murine myocardial macrophage density results from monocyte recruitment and increased hematopoiesis in bone marrow and spleen...
January 16, 2018: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/29331736/dysfunctional-telomeres-and-hematological-disorders
#3
REVIEW
Elena Fiorini, Andrea Santoni, Simona Colla
Telomere biology disorders, which are characterized by telomerase activity haploinsufficiency and accelerated telomere shortening, most commonly manifest as degenerative diseases. Tissues with high rates of cell turnover, such as those in the hematopoietic system, are particularly vulnerable to defects in telomere maintenance genes that eventually culminate in bone marrow (BM) failure syndromes, in which the BM cannot produce sufficient new blood cells. Here, we review how telomere defects induce degenerative phenotypes across multiple organs, with particular focus on how they impact the hematopoietic stem and progenitor compartment and affect hematopoietic stem cell (HSC) self-renewal and differentiation...
January 4, 2018: Differentiation; Research in Biological Diversity
https://www.readbyqxmd.com/read/29323266/aif-loss-deregulates-hematopoiesis-and-reveals-different-adaptive-metabolic-responses-in-bone-marrow-cells-and-thymocytes
#4
Lauriane Cabon, Audrey Bertaux, Marie-Noëlle Brunelle-Navas, Ivan Nemazanyy, Laurianne Scourzic, Laure Delavallée, Laura Vela, Mathieu Baritaud, Sandrine Bouchet, Cécile Lopez, Vu Quang Van, Kevin Garbin, Danielle Chateau, Françoise Gilard, Marika Sarfati, Thomas Mercher, Olivier A Bernard, Santos A Susin
Mitochondrial metabolism is a tightly regulated process that plays a central role throughout the lifespan of hematopoietic cells. Herein, we analyze the consequences of the mitochondrial oxidative phosphorylation (OXPHOS)/metabolism disorder associated with the cell-specific hematopoietic ablation of apoptosis-inducing factor (AIF). AIF-null (AIF-/Y ) mice developed pancytopenia that was associated with hypocellular bone marrow (BM) and thymus atrophy. Although myeloid cells were relatively spared, the B-cell and erythroid lineages were altered with increased frequencies of precursor B cells, pro-erythroblasts I, and basophilic erythroblasts II...
January 11, 2018: Cell Death and Differentiation
https://www.readbyqxmd.com/read/29322685/feasibility-safety-and-tolerance-of-mesenchymal-stem-cell-therapy-for-obstructive-chronic-lung-allograft-dysfunction
#5
Cesar Ariel Keller, Thomas Arthur Gonwa, David Orel Hodge, Derek Joseph Hei, John Michael Centanni, Abba Chedi Zubair
Feasibility, tolerance, and safety of intravenous infusions of allogeneic mesenchymal stem cell (MSC) therapy in lung transplant recipients with bronchiolitis obliterans syndrome (BOS) are not well established. MSCs were manufactured, cryopreserved, transported to our facility, thawed, and infused into nine recipients with moderate BOS (average drop in forced expiratory volume in 1 second was 56.8% ± 3.2% from post-transplant peak) who were refractory to standard therapy and not candidates for retransplant...
January 11, 2018: Stem Cells Translational Medicine
https://www.readbyqxmd.com/read/29321823/yin-and-yang-of-mesenchymal-stem-cells-and-aplastic-anemia
#6
REVIEW
Larisa Broglie, David Margolis, Jeffrey A Medin
Acquired aplastic anemia (AA) is a bone marrow failure syndrome characterized by peripheral cytopenias and bone marrow hypoplasia. It is ultimately fatal without treatment, most commonly from infection or hemorrhage. Current treatments focus on suppressing immune-mediated destruction of bone marrow stem cells or replacing hematopoietic stem cells (HSCs) by transplantation. Our incomplete understanding of the pathogenesis of AA has limited development of targeted treatment options. Mesenchymal stem cells (MSCs) play a vital role in HSC proliferation; they also modulate immune responses and maintain an environment supportive of hematopoiesis...
December 26, 2017: World Journal of Stem Cells
https://www.readbyqxmd.com/read/29311623/basal-p53-expression-is-indispensable-for-mesenchymal-stem-cell-integrity
#7
Siddaraju V Boregowda, Veena Krishnappa, Jacqueline Strivelli, Christopher L Haga, Cori N Booker, Donald G Phinney
Marrow-resident mesenchymal stem cells (MSCs) serve as a functional component of the perivascular niche that regulates hematopoiesis. They also represent the main source of bone formed in adult bone marrow, and their bifurcation to osteoblast and adipocyte lineages plays a key role in skeletal homeostasis and aging. Although the tumor suppressor p53 also functions in bone organogenesis, homeostasis, and neoplasia, its role in MSCs remains poorly described. Herein, we examined the normal physiological role of p53 in primary MSCs cultured under physiologic oxygen levels...
January 8, 2018: Cell Death and Differentiation
https://www.readbyqxmd.com/read/29310643/galega-officinalis-extract-regulate-the-diabetes-mellitus-related-violations-of-proliferation-functions-and-apoptosis-of-leukocytes
#8
Mariia Nagalievska, Mariya Sabadashka, Halyna Hachkova, Nataliia Sybirna
BACKGROUND: An impaired leukocytes function is the factor causing the susceptibility of patients with diabetes mellitus to infections. The outmost importance for the understanding of the immunological processes involved in diabetes pathogenesis is to give the characteritics of the immunological profile and changes therein, during the course of desease. Long-used in folk medicine to treat diabetes Galega officinalis L. has been chosen for the correction of the immune system dysfunction...
January 8, 2018: BMC Complementary and Alternative Medicine
https://www.readbyqxmd.com/read/29305999/high-fat-diet-induced-obesityexacerbateshematopoiesis-deficiency-and-cytopenia-caused-by-5-fluorouracil-via-ppar-%C3%AE
#9
Yanhong Li, Shuai Zhu, Yuanyuan Zhang, Ting Liu, Linchong Su, Qiuping Zhang, Yubin Luo
The present study aims to investigate the influence of high fat diet (HFD) on hematopoietic system recovery under stress condition caused by 5-Fluouracil (5-Fu) and evaluate the alleviating benefit of PPAR-γ inhibition on aggravation of 5-Fu-induced toxicity under HFD condition. Survival rate of HFD or normal diet (ND) mice was monitored after 5-Fu injection. HSC and the progenitor cells in bone marrow were detected at various time points after 5-Fu administration by flow cytometry. Genes expression involved in stem cell and platelet proliferation were tested by RT-PCR...
January 3, 2018: Experimental Hematology
https://www.readbyqxmd.com/read/29288170/atorvastatin-enhances-bone-marrow-endothelial-cell-function-in-corticosteroid-resistant-immune-thrombocytopenia-patients
#10
Yuan Kong, Xie-Na Cao, Xiao-Hui Zhang, Min-Min Shi, Yue-Yun Lai, Yu Wang, Lan-Ping Xu, Ying-Jun Chang, Xiao-Jun Huang
The pathogenesis of corticosteroid-resistant immune thrombocytopenia (ITP), a clinically challenging condition in which patients exhibit either no response to corticosteroids or are corticosteroid-dependent, remains poorly understood. Murine studies suggest that bone marrow (BM) endothelial progenitor cells (EPCs) play a crucial role in regulating megakaryocytopoiesis. However, little is known regarding the number and function of BM EPCs or how to improve impaired BM EPCs in corticosteroid-resistant ITP patients...
December 29, 2017: Blood
https://www.readbyqxmd.com/read/29287914/loss-of-c-kit-function-impairs-arteriogenesis-in-a-mouse-model-of-hindlimb-ischemia
#11
Diana R Hernandez, Adriana Artiles, Juan C Duque, Laisel Martinez, Mariana T Pinto, Keith A Webster, Omaida C Velazquez, Roberto I Vazquez-Padron, Roberta M Lassance-Soares
BACKGROUND: Arteriogenesis is a process whereby collateral vessels remodel usually in response to increased blood flow and/or wall stress. Remodeling of collaterals can function as a natural bypass to alleviate ischemia during arterial occlusion. Here we used a genetic approach to investigate possible roles of tyrosine receptor c-Kit in arteriogenesis. METHODS: Mutant mice with loss of c-Kit function (KitW/W-v), and controls were subjected to hindlimb ischemia. Blood flow recovery was evaluated pre-, post-, and weekly after ischemia...
December 26, 2017: Surgery
https://www.readbyqxmd.com/read/29286581/bone-marrow-features-in-pearson-syndrome-with-neonatal-onset-a-case-report-and-review-of-the-literature
#12
Elisa Tadiotto, Evelina Maines, Daniela Degani, Rita Balter, Andrea Bordugo, Simone Cesaro
Pearson syndrome (PS) is a rare mitochondrial disorder that usually presents with transfusion-dependent macrocytic anemia, exocrine pancreatic dysfunction, and lactic acidosis. Typical bone marrow (BM) features are vacuolization in hematopoietic progenitors, hypocellularity, and ringed sideroblasts. At the neonatal age, PS may have a variable clinical onset. Moreover, there is little information about BM features at this age and the timing of their presentation. We report a neonatal case of PS that presented with refractory anemia and atypical BM features...
December 29, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29285060/inhibitory-effect-of-oxidative-damage-on-cardiomyocyte-differentiation-from-wharton-s-jelly-derived-mesenchymal-stem-cells
#13
Natakarn Nimsanor, Jitrada Phetfong, Chotiros Plabplueng, Kulachart Jangpatarapongsa, Virapong Prachayasittikul, Aungkura Supokawej
Ischemic heart diseases are a serious health problem worldwide. The transplantation of mesenchymal stem cells (MSCs) has been investigated in numerous clinical trials on various other diseases due to the self-renewal capacity of these cells and their potential to differentiate into a variety of cell types. The presence of excess reactive oxygen species in injured myocardium causes cardiac dysfunction and leads to inefficient repair of the heart. The poor outcomes of stem cell transplantation have been suggested to result from residual oxidative damage affecting the transplanted cells...
December 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29283878/occupational-cns-aspergillosis-in-an-immunocompetent-individual-a-diagnostic-challange
#14
Parul Punia, Nidhi Goel, Ishwar Singh, Uma Chaudhary
INTRODUCTION: Immunocompetent individuals are rarely affected by Aspergillus species and its prime importance lies in immunocompromised patients where it can cause disease, ranging from primarily pulmonary infections to dissemination anywhere in the body. Invasive aspergillosis (IA) occurs in patients with risk factors including prolonged neutropenia, neutrophil dysfunction, patient on cytotoxic drugs, steroid therapy, hematological malignancy, AIDS or in patients with bone marrow transplantation...
December 28, 2017: Archives of Environmental & Occupational Health
https://www.readbyqxmd.com/read/29279328/hydrogen-sulfide-inhibits-the-nlrp3-inflammasome-and-reduces-cytokine-production-both-in-vitro-and-in-a-mouse-model-of-inflammation
#15
Mariela Castelblanco, Jérôme Lugrin, Driss Ehirchiou, Sonia Nasi, Isao Ishii, Alexander So, Fabio Martinon, Nathalie Busso
A variety of stimuli, including monosodium urate (MSU) crystals, activate the NLRP3 inflammasome, and this activation involves several molecular mechanisms including xanthine oxidase (XO) upregulation and mitochondrial dysfunction. Upon oligomerization of apoptosis-associated speck-like protein containing a CARD (ASC), caspase-1 becomes active and cleaves the proinflammatory cytokine IL-1β into its active secreted form. Hydrogen sulfide (H2S), a gasotransmitter mainly produced by cystathionine γ-lyase (CSE) in macrophages, could modulate inflammation...
December 26, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29278889/erythromyeloid-derived-trem2-a-major-determinant-of-alzheimer-s-disease-pathology-in-down-syndrome
#16
Ruma Raha-Chowdhury, James W Henderson, Animesh Alexander Raha, Simon R W Stott, Romina Vuono, Simona Foscarin, Liam Wilson, Tiina Annus, Robert Fincham, Kieren Allinson, Vinod Devalia, Robert P Friedland, Anthony Holland, Shahid H Zaman
BACKGROUND: Down syndrome (DS; trisomy 21) individuals have a spectrum of hematopoietic and neuronal dysfunctions, and by the time they reach the age of 40 years, almost all develop Alzheimer's disease (AD) neuropathology which includes senile plaques and neurofibrillary tangles. Inflammation and innate immunity are key players in AD and DS. Triggering receptor expressed in myeloid cells-2 (TREM2) variants have been identified as risk factors for AD and other neurodegenerative diseases...
December 16, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29278309/monotropein-promotes-angiogenesis-and-inhibits-oxidative-stress-induced-autophagy-in-endothelial-progenitor-cells-to-accelerate-wound-healing
#17
Chenggui Wang, Cong Mao, Yiting Lou, Jianxiang Xu, Qingqing Wang, Zengjie Zhang, Qian Tang, Xiaolei Zhang, Huazi Xu, Yongzeng Feng
Attenuating oxidative stress-induced damage and promoting endothelial progenitor cell (EPC) differentiation are critical for ischaemic injuries. We suggested monotropein (Mtp), a bioactive constituent used in traditional Chinese medicine, can inhibit oxidative stress-induced mitochondrial dysfunction and stimulate bone marrow-derived EPC (BM-EPC) differentiation. Results showed Mtp significantly elevated migration and tube formation of BM-EPCs and prevented tert-butyl hydroperoxide (TBHP)-induced programmed cell death through apoptosis and autophagy by reducing intracellular reactive oxygen species release and restoring mitochondrial membrane potential, which may be mediated viamTOR/p70S6K/4EBP1 and AMPK phosphorylation...
December 26, 2017: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/29275062/intravenous-infusion-of-bone-marrow-derived-mesenchymal-stem-cells-reduces-erectile-dysfunction-following-cavernous-nerve-injury-in-rats
#18
Yohei Matsuda, Masanori Sasaki, Yuko Kataoka-Sasaki, Akio Takayanagi, Ko Kobayashi, Shinichi Oka, Masahito Nakazaki, Naoya Masumori, Jeffery D Kocsis, Osamu Honmou
INTRODUCTION: Intravenous preload (delivered before cavernous nerve [CN] injury) of bone marrow-derived mesenchymal stem cells (MSCs) can prevent or decrease postoperative erectile dysfunction (J Sex Med 2015;12:1713-1721). In the present study, the potential therapeutic effects of intravenously administered MSCs on postoperative erectile dysfunction were evaluated in a rat model of CN injury. METHODS: Male Sprague-Dawley rats were randomized into 2 groups after electric CN injury...
December 20, 2017: Sexual Medicine
https://www.readbyqxmd.com/read/29262476/-short-stature-optic-nerve-atrophy-and-pelger-hu%C3%A3-t-anomaly-syndrome-with-antibody-immunodeficiency-and-aplastic-anemia-a-case-report-and-literature-review
#19
T Y He, N Zhang, Y Xia, Y Luo, C R Li, J Yang
Objective: To investigate the clinical features and genetic characteristics of cases with NBAS gene defects. Method: Characteristics of clinical materials, immunological data and gene mutation of the first case in China with NBAS gene mutation were retrospectively analyzed. The related literature was searched by using search terms'NBAS'. Result: A 2-year-four-month old girl, was admitted due to 'fever and pallor for one day'. There was an intrauterine growth retardation at her fetal stage. Since her birth, she had suffered from recurrent infections and development delay was accompanied by persistent liver dysfunction...
December 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29242293/variable-impairment-of-platelet-functions-in-patients-with-severe-genetically-linked-immune-deficiencies
#20
Magdolna Nagy, Tom G Mastenbroek, Nadine J A Mattheij, Susanne de Witt, Kenneth J Clemetson, Janbernd Kirschner, Ansgar S Schulz, Thomas Vraetz, Carsten Speckmann, Attila Braun, Judith M E M Cosemans, Barbara Zieger, Johan W M Heemskerk
In patients with dysfunctions of the Ca2+ channel ORAI1, stromal interaction molecule 1 (STIM1) or integrin-regulating kindlin-3 (FERMT3), severe immunodeficiency is frequently linked to abnormal platelet activity. In this paper, we studied in nine rare patients, including relatives, with confirmed genetic mutations of ORAI1, STIM1 or FERMT3, platelet responsiveness by multi-parameter assessment of whole blood thrombus formation under high-shear flow conditions. In platelets isolated from 5 out of 6 patients with ORAI1 or STIM1 mutations, store-operated Ca2+ entry (SOCE) was (in)completely defective compared to control platelets...
December 14, 2017: Haematologica
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