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https://www.readbyqxmd.com/read/29773597/hematopoietic-stem-cell-loss-and-hematopoietic-failure-in-severe-aplastic-anemia-is-driven-by-macrophages-and-aberrant-podoplanin-expression
#1
Amanda McCabe, Julianne N P Smith, Angelica Costello, Jackson Maloney, Divya Katikaneni, Katherine C MacNamara
Severe aplastic anemia results from profound hematopoietic stem cell loss. T cells and interferon gamma have long been associated with severe aplastic anemia, yet the underlying mechanisms driving hematopoietic stem cell loss remain unknown. Using a mouse model of severe aplastic anemia, we demonstrate that interferon gamma-dependent hematopoietic stem cell loss required macrophages. Interferon gamma was necessary for bone marrow macrophage persistence, despite loss of other myeloid cells and hematopoietic stem cells...
May 17, 2018: Haematologica
https://www.readbyqxmd.com/read/29772350/unmanipulated-haploidentical-hematopoietic-stem-cell-transplantation-achieved-outcomes-comparable-with-matched-unrelated-donor-transplantation-in-young-acquired-severe-aplastic-anemia
#2
Yue Lu, Rui-Juan Sun, Yan-Li Zhao, Min Xiong, Xing-Yu Cao, Jing-Pin Zhang, Zhi-Jie Wei, Jia-Rui Zhou, De-Yan Liu, Dao-Pei Lu
Salvage haploidentical HSCT is considered in patients with severe aplastic anemia (SAA) if matched unrelated donor (MUD) is unavailable. However, studies on haplo- and MUD transplantation in SAA are yet lacking. The present study retrospectively analyzed the outcomes of 89 young SAA patients who underwent unmanipulated alternative hematopoietic stem cell transplantation (HSCT) between September 2012 and September 2016 at our single center. 41 patients received haploidentical donors, and 48 patients received MUD for HSCT...
May 14, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29749397/recurrent-somatic-mutations-are-rare-in-patients-with-cryptic-dyskeratosis-congenita
#3
Martin Kirschner, Angela Maurer, Marcin W Wlodarski, Monica S Ventura Ferreira, Anne-Sophie Bouillon, Insa Halfmeyer, Wolfgang Blau, Michael Kreuter, Martin Rosewich, Selim Corbacioglu, Joachim Beck, Michaela Schwarz, Jörg Bittenbring, Markus P Radsak, Christian Matthias Wilk, Steffen Koschmieder, Matthias Begemann, Ingo Kurth, Mirle Schemionek, Tim H Brümmendorf, Fabian Beier
Dyskeratosis congenita (DKC) is a paradigmatic telomere disorder characterized by substantial and premature telomere shortening, bone marrow failure, and a dramatically increased risk of developing myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML). DKC can occur as a late-onset, so-called cryptic form, with first manifestation in adults. Somatic MDS-related mutations are found in up to 35% of patients with acquired aplastic anemia (AA), especially in patients with short telomeres. The aim of our study was to investigate whether cryptic DKC is associated with an increased incidence of MDS-related somatic mutations, thereby linking the accelerated telomere shortening with the increased risk of MDS/AML...
April 2, 2018: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/29720492/sustained-clonal-hematopoiesis-by-hla-lacking-hematopoietic-stem-cells-without-driver-mutations-in-aplastic-anemia
#4
Tatsuya Imi, Takamasa Katagiri, Kazuyoshi Hosomichi, Yoshitaka Zaimoku, Viet Hoang Nguyen, Noriharu Nakagawa, Atsushi Tajima, Tetsuichi Yoshizato, Seishi Ogawa, Shinji Nakao
Clonal hematopoiesis by hematopoietic stem progenitor cells (HSPCs) that lack an HLA class I allele (HLA- HSPCs) is common in patients with acquired aplastic anemia (AA); however, it remains unknown whether the cytotoxic T lymphocyte (CTL) attack that allows for survival of HLA- HSPCs is directed at nonmutated HSPCs or HSPCs with somatic mutations or how escaped HLA- HSPC clones support sustained hematopoiesis. We investigated the presence of somatic mutations in HLA- granulocytes obtained from 15 AA patients in long-term remission (median, 13 years; range, 2-30 years)...
May 8, 2018: Blood Advances
https://www.readbyqxmd.com/read/29713553/acquired-amegakaryocytic-thrombocytopenia-and-pure-red-cell-aplasia-in-thymoma
#5
Sumit Dahal, Eliza Sharma, Suyash Dahal, Binav Shrestha, Bikash Bhattarai
Association of thymoma with myasthenia gravis, pure red cell aplasia, and aplastic anemia is well documented. However, thymoma complicated by acquired amegakaryocytic thrombocytopenia (AAMT) is rarely reported. Here, we present a case of a 60-year-old male with past medical history of recurrent invasive thymoma who presented with cough and blood in sputum. He was found to have severe normocytic normochromic anemia and thrombocytopenia that did not improve with intravenous steroids or multiple transfusions of red cells and platelets...
2018: Case Reports in Hematology
https://www.readbyqxmd.com/read/29703819/new-insights-from-studies-of-clonal-hematopoiesis
#6
Christopher J Gibson, David P Steensma
Clonal hematopoiesis (CH) describes an asymptomatic expansion of blood cells descended from a single hematopoietic stem cell. Recent studies have shown that CH increases in frequency with aging, and is often driven by somatic mutations in genes that are recurrently mutated in hematologic malignancies. When CH is associated with a mutation in a leukemia-associated gene at a variant allele frequency of 0.02 or greater, it is termed "clonal hematopoiesis of indeterminate potential" (CHIP).  CHIP has a 0...
April 27, 2018: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/29663189/a-characteristic-flow-cytometric-pattern-with-broad-forward-scatter-and-narrowed-side-scatter-helps-diagnose-immune-thrombocytopenia-itp
#7
Raita Araki, Ryosei Nishimura, Rie Kuroda, Toshihiro Fujiki, Shintaro Mase, Kazuhiro Noguchi, Yasuhiro Ikawa, Hideaki Maeba, Akihiro Yachie
Various disorders cause severe thrombocytopenia, which can lead to critical hemorrhage. Procedures that rapidly support the diagnosis and risk factors for serious bleeding were explored, with a focus on immune thrombocytopenia (ITP). Twenty-five patients with thrombocytopenia, including 13 with newly diagnosed ITP, 3 with chronic ITP, 6 with aplastic anemia (AA), and 3 with other thrombocytopenia (one acute myeloid leukemia, one acute lymphoblastic leukemia, and one hemophagocytic lymphohistiocytosis), were reviewed...
April 16, 2018: International Journal of Hematology
https://www.readbyqxmd.com/read/29564940/quantitative-measurement-of-oxidative-damage-in-erythrocytes-as-indicator-in-benzene-intoxications
#8
José Luis Martínez-Rodríguez, Rosalinda Gutiérrez-Hernández, Claudia Araceli Reyes-Estrada, Angelica Judith Granados-López, Tatiana Arcos-Ortega, Jesús Adrián López
The metabolism of aromatic hydrocarbons by the organism forms products that cause cell death depending on the type of exposure. Benzene exposure has been linked to oxidative stress, hepatic damage, aplastic anemia and hematopoietic cancer as lymphoid and myeloid leukemia. However, there are not fast methods to evaluate chronically benzene exposure in human blood. The objective of this work was the evaluation of the correlation between oxidative damage with benzene exposure and the level of Cellular Plasma Membrane Stability (CPMS) in erythrocytes to use it as a future indicator to determine the grade of benzene intoxications...
March 22, 2018: Toxicology Mechanisms and Methods
https://www.readbyqxmd.com/read/29532539/cotransplantation-of-haploidentical-hematopoietic-stem-cells-and-allogeneic-bone-marrow-derived-mesenchymal-stromal-cells-as-a-first-line-treatment-in-very-severe-aplastic-anemia-patients-with-refractory-infections
#9
Chunyan Yue, Yingying Ding, Yang Gao, Li Li, Yan Pang, Zenghui Liu, Hang Zhang, Yang Xiao, Zujun Jiang, Haowen Xiao
OBJECTIVES: In patients with very severe aplastic anemia (VSAA), neutropenia is prolonged and persistent, resulting in refractory overwhelming infections. Hematopoiesis recovery is urgently needed. METHODS: Six patients with de novo VSAA lacking HLA-identical sibling donors and those who experienced refractory infections underwent haploidentical related donor (HRD) hematopoietic stem cell transplantation (HSCT) as a first-line therapy. The conditioning regimen consisted of busulfan, cyclophosphamide, and rabbit antithymocyte globulin...
March 12, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29467192/combination-of-the-low-anticoagulant-heparin-cx-01-with-chemotherapy-for-the-treatment-of-acute-myeloid-leukemia
#10
Tibor J Kovacsovics, Alice Mims, Mohamed E Salama, Jeremy Pantin, Narayanam Rao, Ken M Kosak, Peter Ahorukomeye, Martha J Glenn, Michael W N Deininger, Kenneth M Boucher, Linda M Bavisotto, Gerardo Gutierrez-Sanchez, Thomas P Kennedy, Stephen G Marcus, Paul J Shami
Relapses in acute myelogenous leukemia (AML) are a result of quiescent leukemic stem cells (LSCs) in marrow stromal niches, where they resist chemotherapy. LSCs employ CXCL12/CXCR4 to home toward protective marrow niches. Heparin disrupts CXCL12-mediated sequestration of cells in the marrow. CX-01 is a low-anticoagulant heparin derivative. In this pilot study, we combined CX-01 with chemotherapy for the treatment of AML. Induction consisted of cytarabine and idarubicin (7 + 3) with CX-01. Twelve patients were enrolled (median age, 56 years; 3 women)...
February 27, 2018: Blood Advances
https://www.readbyqxmd.com/read/29449166/clinical-characteristics-and-treatment-outcomes-of-pulmonary-invasive-fungal-infection-among-adult-patients-with-hematological-malignancy-in-a-medical-centre-in-taiwan-2008-2013
#11
Chien-Yuan Chen, Wang-Huei Sheng, Feng-Ming Tien, Po-Chu Lee, Shang-Yi Huang, Jih-Luh Tang, Woei Tsay, Hwei-Fang Tien, Po-Ren Hsueh
BACKGROUND/PURPOSE: This study was aimed to investigate clinical characteristics and treatment outcomes of pulmonary invasive fungal infection (IFI) among patients with hematological malignancy. METHODS: All patients with hematological malignancy who were treated at a medical centre from 2008 to 2013 were evaluated. Pulmonary IFI was classified according to the European Organization for Research and Treatment of Cancer 2008 consensus. RESULTS: During the study period, 236 (11...
January 31, 2018: Journal of Microbiology, Immunology, and Infection, Wei Mian Yu Gan Ran za Zhi
https://www.readbyqxmd.com/read/29416752/fanconi-anemia-germline-variants-as-susceptibility-factors-in-aplastic-anemia-mds-and-aml
#12
Bartlomiej Przychodzen, Hideki Makishima, Mikkael A Sekeres, Suresh Kumar Balasubramanian, Swapna Thota, Bhumika J Patel, Michael Clemente, Cassandra Hirsch, Brittney Dienes, Jaroslaw P Maciejewski
Using next generation sequencing we have systematically analyzed a large cohort of 489 patients with bone marrow failure (BMF), including myelodysplastic syndrome (MDS), acute myeloid leukemia (AML), aplastic anemia (AA), and related conditions for the presence of germline (GL) alterations in Fanconi Anemia (FA) and telomerase genes. We have detected an increased frequency of heterozygous FA gene mutations in MDS and to lesser degree in AML suggesting that the presence of one normal allele may not be completely protective and indeed heterozygous FA lesions may have a long latency period before hematologic manifestation...
January 5, 2018: Oncotarget
https://www.readbyqxmd.com/read/29374141/ipsc-modeling-of-severe-aplastic-anemia-reveals-impaired-differentiation-and-telomere-shortening-in-blood-progenitors
#13
Dario Melguizo-Sanchis, Yaobo Xu, Dheraj Taheem, Min Yu, Katarzyna Tilgner, Tomas Barta, Katja Gassner, George Anyfantis, Tengfei Wan, Ramu Elango, Sameer Alharthi, Ashraf A El-Harouni, Stefan Przyborski, Soheir Adam, Gabriele Saretzki, Sujith Samarasinghe, Lyle Armstrong, Majlinda Lako
Aplastic Anemia (AA) is a bone marrow failure (BMF) disorder, resulting in bone marrow hypocellularity and peripheral pancytopenia. Severe aplastic anemia (SAA) is a subset of AA defined by a more severe phenotype. Although the immunological nature of SAA pathogenesis is widely accepted, there is an increasing recognition of the role of dysfunctional hematopoietic stem cells in the disease phenotype. While pediatric SAA can be attributable to genetic causes, evidence is evolving on previously unrecognized genetic etiologies in a proportion of adults with SAA...
January 26, 2018: Cell Death & Disease
https://www.readbyqxmd.com/read/29344583/heterozygous-rtel1-variants-in-bone-marrow-failure-and-myeloid-neoplasms
#14
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/29344089/bone-marrow-failure-may-be-caused-by-chromosome-anomalies-exerting-effects-on-runx1t1-gene
#15
R Valli, L Vinti, A Frattini, M Fabbri, G Montalbano, C Olivieri, A Minelli, F Locatelli, F Pasquali, E Maserati
Background: The majority of the cases of bone marrow failure syndromes/aplastic anaemias (BMFS/AA) are non-hereditary and considered idiopathic (80-85%). The peripheral blood picture is variable, with anaemia, neutropenia and/or thrombocytopenia, and the patients with idiopathic BMFS/AA may have a risk of transformation into a myelodysplastic syndrome (MDS) and/or an acute myeloid leukaemia (AML), as ascertained for all inherited BMFS. We already reported four patients with different forms of BMFS/AA with chromosome anomalies as primary etiologic event: the chromosome changes exerted an effect on specific genes, namely RUNX1, MPL, and FLI1, leading to the disease...
2018: Molecular Cytogenetics
https://www.readbyqxmd.com/read/29334367/unmanipulated-haploidentical-transplantation-conditioning-with-busulfan-cyclophosphamide-and-anti-thymoglobulin-for-adult-severe-aplastic-anaemia
#16
L-P Xu, Z-L Xu, F-R Wang, X-D Mo, T-T Han, W Han, Y-H Chen, Y-Y Zhang, J-Z Wang, Y Wang, C-H Yan, Y-Q Sun, F-F Tang, X-H Zhang, X-J Huang
We conducted a retrospective analysis to evaluate outcomes of haploidentical transplantation in adult severe aplastic anaemia (SAA) patients. Fifty-one adults received haploidentical transplantation between May 2011 and December 2016. Patients were administered busulfan (Bu), cyclophosphamide (Cy) and anti-thymoglobulin (ATG) as conditioning regimens, followed by bone marrow and peripheral blood transplantation. The patients' median age was 25 years. Forty-nine patients survived for more than 28 days and all achieved donor myeloid engraftment...
February 2018: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/29296774/treated-secondary-acute-myeloid-leukemia-a-distinct-high-risk-subset-of-aml-with-adverse-prognosis
#17
Prajwal Boddu, Hagop M Kantarjian, Guillermo Garcia-Manero, Farhad Ravandi, Srdan Verstovsek, Elias Jabbour, Gautam Borthakur, Marina Konopleva, Kapil N Bhalla, Naval Daver, Courtney D DiNardo, Christopher B Benton, Koichi Takahashi, Zeev Estrov, Sherry R Pierce, Michael Andreeff, Jorge E Cortes, Tapan M Kadia
Secondary acute myeloid leukemia (s-AML) includes therapy-related AML and AML evolving from antecedent hematological disorder (AHD). s-AML arising after treating AHD likely represents a prognostically distinct, high-risk disease category. In this study, treated s-AML (ts-AML) was defined by: (1) prior diagnosis of myelodysplasia, myeloproliferative neoplasm, or aplastic anemia and (2) at least 1 therapy for that diagnosis. ts-AML was categorized by age (< or ≥60 years), and each cohort assessed for response rates and overall survival (OS) on various treatment regimens...
July 25, 2017: Blood Advances
https://www.readbyqxmd.com/read/29243948/shanghai-health-study-2001-2009-what-was-learned-about-benzene-health-effects
#18
Sherilyn A Gross, Dennis J Paustenbach
The Shanghai Health Study (SHS) was a large epidemiology study conducted as a joint effort between the University of Colorado and Fudan University in Shanghai, China. The study was funded by members of the American Petroleum Institute between 2001 and 2009 and was designed to evaluate the human health effects associated with benzene exposure. Two arms of the SHS included: an occupational-based molecular epidemiology study and several hospital-based case control studies. Consistent with historical literature, following sufficient exposure to relatively high airborne concentrations and years of exposure, the SHS concluded that exposure to benzene resulted in an increased risk of various blood and bone marrow abnormalities such as benzene poisoning, aplastic anemia (AA), myelodysplastic syndrome (MDS), and acute myeloid leukemia (AML)...
March 2018: Critical Reviews in Toxicology
https://www.readbyqxmd.com/read/29222240/old-and-new-tools-in-the-clinical-diagnosis-of-inherited-bone-marrow-failure-syndromes
#19
REVIEW
Allison H West, Jane E Churpek
Patients with inherited bone marrow failure syndromes (IBMFSs) classically present with specific patterns of cytopenias along with congenital anomalies and/or other physical features that are often recognizable early in life. However, increasing application of genomic sequencing and clinical awareness of subtle disease presentations have led to the recognition of IBMFS in pediatric and adult populations more frequently than previously realized, such as those with early onset myelodysplastic syndrome (MDS). Given the well-defined differences in clinical management needs and outcomes for aplastic anemia, acute myeloid leukemia, and MDS in patients with an IBMFS vs those occurring sporadically, as well as nonhematologic comorbidities in patients with IBMFSs, it is critical for hematologists to understand how to approach screening for the currently known IBMFSs...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29140934/response-of-symptomatic-persistent-chronic-disseminated-candidiasis-to-corticosteroid-therapy-in-immunosuppressed-pediatric-patients-case-study-and-review-of-the-literature
#20
Vered Shkalim-Zemer, Itzhak Levi, Salvador Fischer, Hannah Tamary, Joanne Yakobovich, Gali Avrahami, Gil Gilad, Sara Elitzur, Isaac Yaniv, Ronit Elhasid, Michal Manistersky, Itamar Shalit
BACKGROUND: Chronic disseminated candidiasis (CDC) is a severe invasive fungal infection principally observed during neutrophil recovery in patients with acute leukemia treated with intensive chemotherapy. Its pathophysiology remains unclear. We describe the management of six children with symptomatic CDC who did not respond to antifungal therapy. METHODS: The databases of the hematology-oncology departments of two tertiary pediatric medical centers were searched for all patients diagnosed with CDC from 2003 to 2015 who responded to corticosteroids after failing antifungal therapy...
November 14, 2017: Pediatric Infectious Disease Journal
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