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Aplastic anemia

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https://www.readbyqxmd.com/read/28729300/rapamycin-is-highly-effective-in-murine-models-of-immune-mediated-bone-marrow-failure
#1
Xingmin Feng, Zenghua Lin, Wanling Sun, Maile K Hollinger, Marie J Desierto, Keyvan Keyvanfar, Daniela Malide, Pawel Muranski, Jichun Chen, Neal S Young
Acquired aplastic anemia, the prototypical bone marrow failure disease, is characterized by pancytopenia and marrow hypoplasia. Most aplastic anemia patients respond to immunosuppressive therapy, usually as anti-thymocyte globulin and cyclosporine, but some relapse on cyclosporine withdrawal or require long-term administration of cyclosporine to maintain blood counts. In the current study, we tested efficacy of rapamycin as a new or alternative treatment in mouse models of immune-mediated bone marrow failure...
July 20, 2017: Haematologica
https://www.readbyqxmd.com/read/28728504/the-diverse-expression-of-the-wt1-gene-in-patients-with-acquired-bone-marrow-failure-syndromes
#2
Yahong You, Jiali Huo, Shihong Lu, Yingqi Shao, Meili Ge, Jun Shi, Xingxin Li, Jinbo Huang, Zhendong Huang, Jing Zhang, Min Wang, Neng Nie, Yizhou Zheng
Acquired bone marrow failure syndromes (aBMFS) encompass a wide range of diseases. A study to investigate WT1 expression in BM was conducted in 387 patients with aBMFS in China. The WT1 level in patients with aplastic anemia (AA) was significantly lower than that in patients with paroxysmal nocturnal hemoglobinuria (PNH, p = .023) and myelodysplastic syndrome (MDS, p < .001). In addition, the WT1 level in patients with MDS significantly increased as the disease progressed to an advanced stage. Patients with hypoplastic MDS had a differentiated expression level of WT1 compared with that of NSAA (p < ...
July 21, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28728262/-evaluation-of-efficacy-of-immunosuppressive-therapy-plus-recombinant-human-thrombopoietin-for-children-with-severe-aplastic-anemia
#3
K Zhou, C X Liu, Y Li, J P Li, H H Fan, L Zhang, L P Jing, G X Peng, L Ye, Y Li, L Song, X Zhao, W R Yang, Z J Wu, F Chen, F K Zhang
Objective: To evaluate the therapeutic efficacy and safety of immunosuppressive therapy (IST) combined with recombinant human thrombopoietin (rhTPO) for severe aplastic anemia (SAA) in pediatric patients. Method: A retrospective case-control study was conducted and the clinical data of 45 pediatric patients with de novo SAA admitted to the Anemia Diagnosis and Treatment Center of Chinese Academy of Medical Sciences & Blood Disease Hospital during the period from December 2009 to December 2014 were analyzed...
July 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28715625/-myelofibrosis-in-a-benzene-exposed-cleaning-worker
#4
Roser Bausà, Lydia Navarro, Imma Cortès-Franch
Long-term exposure to benzene has been associated with several blood malignancies, including aplastic anemia, myeloproliferative neoplasms, and different leukemias. We present a case of primary myelofibrosis in a 59-year-old woman who worked as a cleaner at a car dealership and automobile mechanic shop. For 25 years, she used gasoline as a degreaser and solvent to clean engine parts, floors and work desks on a daily basis. She was referred by her primary care provider to the Occupational Health Unit of Barcelona to assess whether her illness was work-related...
July 2017: Archivos de Prevención de Riesgos Laborales
https://www.readbyqxmd.com/read/28713749/mucormycosis-of-the-frontal-sinus-a-rare-case-report-and-review
#5
Nanda Kishore Sahoo, Vishal Kulkarni, Amit K Bhandari, Arun Kumar
Mucormycosis of the frontal sinus are rarely seen in day to day clinical practice. Although this fungus is commonly found in the environment, the disease is usually prevented by the immune system and is hence rare. Well-recognized risk factors for the disease include diabetes mellitus, leukemia, aplastic anemia, myelodysplastic syndrome, blood dyscrasias, and immunosuppressive therapy in organ transplantation, renal disease, sepsis, and severe burns. The disease is primarily found in those who are immunocompromised, but it may also manifest in immuno competent persons...
January 2017: Annals of Maxillofacial Surgery
https://www.readbyqxmd.com/read/28711931/apc-c-is-essential-for-hematopoiesis-and-impaired-in-aplastic-anemia
#6
Jia Wang, Min-Zhi Yin, Ke-Wen Zhao, Fang Ke, Wen-Jie Jin, Xiao-Lin Guo, Tian-Hui Liu, Xiao-Ye Liu, Hao Gu, Xiao-Min Yu, Zhen Li, Li-Li Mu, Deng-Li Hong, Jing Chen, Guo-Qiang Chen
Anaphase promoting complex/cyclosome (APC/C) is essential for cell cycle progression. Recently, its non-mitotic functions were also reported but less studied in several tissues including hematopoietic cells. Here, we developed an inducible Anapc2 (a core subunit of APC/C) knockout mice. The animals displayed a fatal bone marrow failure within 7 days after knockout induction. Their hematopoietic stem and progenitor cells (HSPCs) demonstrated a sharp decline and could form little colony. Further, the results of BrdU label-retaining cell assay showed that the dormant HPSCs lost rapidly...
June 28, 2017: Oncotarget
https://www.readbyqxmd.com/read/28706392/acquired-aplastic-anemia-associated-with-trisomy-eight-converting-into-acute-myeloid-leukemia
#7
Sumit Grover, Amit Kumar Dhiman, Bhavna Garg, Neena Sood, Vikram Narang
Aplastic anemia (AA) is nowadays considered to be a clonal disorder arising from a defective hematopoietic stem cell developing after a generalized insult to bone marrow. Immunosuppressive treatment (IST) of AA causes suppression of the target dominant population of haematopoietic cells allowing the defective non targeted clones to expand. This may give rise to acute leukemia. Cytogenetic studies for chromosomal aberrations such as trisomy and monosomy may help in detecting such conversions. We present a case of acquired AA in a 60-year-old male presenting with pancytopenia and hypoplastic marrow treated with antithymocyte globulin, converting into myelodysplastic syndrome and later on acute promyelocytic leukemia after being in remission for 4 years...
July 2017: Journal of Laboratory Physicians
https://www.readbyqxmd.com/read/28705902/long-term-risk-of-cancer-development-in-adult-patients-with-idiopathic-aplastic-anemia-after-treatment-with-anti-thymocyte-globulin
#8
Joost Gk van der Hem, Liesbeth C de Wreede, Anneke Brand, Hendrik Veelken, Jh Frederik Falkenburg, Constantijn Jm Halkes
No abstract text is available yet for this article.
July 13, 2017: Haematologica
https://www.readbyqxmd.com/read/28705540/three-year-safety-of-radium-223-dichloride-in-patients-with-castration-resistant-prostate-cancer-and-symptomatic-bone-metastases-from-phase-3-randomized-alpharadin-in-symptomatic-prostate-cancer-trial
#9
Christopher C Parker, Robert E Coleman, Oliver Sartor, Nicholas J Vogelzang, David Bottomley, Daniel Heinrich, Svein I Helle, Joe M O'Sullivan, Sophie D Fosså, Aleš Chodacki, Paweł Wiechno, John Logue, Mihalj Seke, Anders Widmark, Dag Clement Johannessen, Peter Hoskin, Nicholas D James, Arne Solberg, Isabel Syndikus, Jan Kliment, Steffen Wedel, Sibylle Boehmer, Marcos Dall'Oglio, Lars Franzén, Øyvind S Bruland, Oana Petrenciuc, Karin Staudacher, Rui Li, Sten Nilsson
BACKGROUND: In Alpharadin in Symptomatic Prostate Cancer (ALSYMPCA) trial, radium-223 versus placebo prolonged overall survival with favorable safety in castration-resistant prostate cancer patients with symptomatic bone metastases. Long-term radium-223 monitoring underlies a comprehensive safety and risk/benefit assessment. OBJECTIVE: To report updated ALSYMPCA safety, including long-term safety up to 3 yr after the first injection. DESIGN, SETTING, AND PARTICIPANTS: Safety analyses from phase 3 randomized ALSYMPCA trial included patients receiving ≥1 study-drug injection (600 radium-223 and 301 placebo)...
July 10, 2017: European Urology
https://www.readbyqxmd.com/read/28696061/outcomes-and-healthcare-utilization-in-children-and-young-adults-with-aplastic-anemia-a-multiinstitutional-analysis
#10
Ashish Gupta, Pingfu Fu, Hasan Hashem, Anant Vatsayan, Steven Shein, Jignesh Dalal
BACKGROUND: Aplastic anemia is a bone marrow failure syndrome with high mortality affecting children and young adults. Although current treatment guidelines recommend hematopoietic stem cell transplant (HCT) for patients with matched sibling donors, outcomes with alternate donor options have been improving. PROCEDURE: We analyzed a validated multiinstitutional pediatric cohort using one of the largest pediatric and young adult database, the Pediatric Health Information System, for patients diagnosed with aplastic anemia (AA) from 2006 to 2015...
July 11, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28695971/transfusion-requirements-following-total-knee-arthroplasty-in-patients-with-preexisting-blood-dyscrasia
#11
Martin Roche, Tsun Yee Law, Samuel Rosas, Augustus J Rush, Anton Khlopas, Morad Chughtai, Michael A Mont
INTRODUCTION: There is a paucity of studies investigating the incidence of transfusions in various blood dyscrasias after total knee arthroplasty (TKA). The purpose of this study was: 1) to compare the incidence of transfusion post-TKA in patients with and without blood dyscrasia; 2) to compare the post-TKA transfusion incidence of patients with the various individual blood dyscrasias; and 3) to stratify the findings based on gender and age. MATERIALS AND METHODS: A retrospective review of a Medicare database for patients with preexisting blood dyscrasia was conducted...
July 11, 2017: Surgical Technology International
https://www.readbyqxmd.com/read/28692030/hla-mismatched-bone-marrow-transplantation-in-severe-aplastic-anemia
#12
L S Bachegowda, M V Shah, L W Veltri, A Tanase, U Popat, P Anderlini, J Chen, G Rondon, R E Champlin, S O Ciurea
No abstract text is available yet for this article.
July 10, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28683082/the-herbal-decoction-modified-danggui-buxue-tang-attenuates-immune-mediated-bone-marrow-failure-by-regulating-the-differentiation-of-t-lymphocytes-in-an-immune-induced-aplastic-anemia-mouse-model
#13
Peiying Deng, Xue Li, Yi Wei, Juan Liu, Meng Chen, Yamei Xu, Bin Dong, Lingqun Zhu, Limin Chai
Angelicae Sinensis, Radix Astragali and Rhizoma Coptidis are all herbs of modified Danggui Buxue Tang (DGBX) and are extensively applied herbs in traditional Chinese medicine for the treatment of anemia and inflammation. In this study, immune-induced AA mice were used as an animal model, and the immunosuppressive agent, Ciclosporin A (CsA), was used as a positive control. Multiple pro-inflammatory cytokines were examined by bead-based multiplex flow cytometry. The T-cell subsets were assessed using a fluorescence-activated cell sorter (FACS)...
2017: PloS One
https://www.readbyqxmd.com/read/28679999/bone-marrow-failure-in-childhood-central-pathology-review-of-a-nationwide-registry
#14
Masafumi Ito
Refractory cytopenia of childhood (RCC) was proposed as a provisional entity in the 2008 WHO classification of myelodysplastic syndromes (MDS). It is defined as a childhood MDS featuring persistent cytopenia without increase blasts in bone marrow (BM) or peripheral blood (PB). Because the majority of RCC cases feature hypocellularity and pancytopenia, it is quite challenging to differentiate RCC from acquired aplastic anemia (AA) and many kinds of inherited bone marrow failure syndromes (IBMFS). Diagnosis of RCC requires BM histology of characteristic features such as isolated erythroid islet with left shift, abnormal localization and micromegakaryocytes...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28677684/cotransplantation-of-bone-marrow-derived-mesenchymal-stem-cells-in-haploidentical-hematopoietic-stem-cell-transplantation-in-patients-with-severe-aplastic-anemia-an-interim-summary-for-a-multicenter-phase-ii-trial-results
#15
Z Liu, Y Zhang, H Xiao, Z Yao, H Zhang, Q Liu, B Wu, D Nie, Y Li, Y Pang, Z Fan, L Li, Z Jiang, F Duan, H Li, P Zhang, Y Gao, L Ouyang, C Yue, M Xie, C Shi, Y Xiao, S Wang
This corrects the article DOI: 10.1038/bmt.2016.347.
July 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28675769/outcomes-of-allogeneic-stem-cell-transplantation-in-patients-with-paroxysmal-nocturnal-hemoglobinuria-with-or-without-aplastic-anemia
#16
Sung-Eun Lee, Sung Soo Park, Young-Woo Jeon, Jae-Ho Yoon, Byung-Sik Cho, Ki-Sung Eom, Yoo-Jin Kim, Seok Lee, Chang-Ki Min, Hee-Je Kim, Seok-Goo Cho, Dong-Wook Kim, Woo-Sung Min, Jong Wook Lee
OBJECTIVE: The aim of this study was to evaluate the long-term outcomes of allogeneic stem cell transplantation (SCT) in patients with paroxysmal nocturnal hemoglobinuria (PNH) with or without aplastic anemia (AA). METHOD: A total of 33 patients with PNH clones who underwent allogeneic SCT were analyzed. RESULTS: After a median follow-up of 57 months (range, 6.0-151.3), the 5-year estimated overall survival rate was 87.9 ± 5.7%. Four patients died of transplant-related mortality (TRM)...
July 4, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28664500/trend-and-treatment-patterns-of-aplastic-anemia-in-korea-pure-red-cell-aplasia-and-myelodysplastic-syndrome-in-korea-a-nation-wide-analysis
#17
Yunsuk Choi, Jae-Cheol Jo, Hee-Jeong Jeon, Dong Wook Kim, Myung Hee Chang, Hawk Kim
Aplastic anemia (AA) and pure red cell aplasia (PRCA) appear to be more prevalent in Asian countries including Korea. However, there are no exact data regarding its prevalence and frequency of allogeneic hematopoietic cell transplantation (HCT) in Korea. Here, we present demographic data relating to AA/PRCA/MDS in Korea. Data were prepared by retrieval from a computerized database maintained by the National Health Insurance Service and Korea National Statistical Office. HCT data were collected from all HCT centers in Korea...
June 29, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28655810/antithymocyte-globulin-and-transplants-for-aplastic-anemia
#18
EDITORIAL
Andrea Bacigalupo
No abstract text is available yet for this article.
July 2017: Haematologica
https://www.readbyqxmd.com/read/28653480/eltrombopag-for-secondary-failure-of-platelet-recovery-post-allogeneic-hematopoietic-stem-cell-transplant-in-children
#19
Salah Ali, Adam Gassas, Melanie Kirby-Allen, Joerg Krueger, Muhammad Ali, Tal Schechter
Secondary failure of platelet engraftment occurs in 20% of patients undergoing allogeneic HSCT and is associated with poor outcome. Currently, there are no guidelines for treatment of late thrombocytopenia and platelet transfusion is the mainstay of treatment. Here, we describe the use of Eltrombopag to treat secondary failure of platelet recovery following HSCT in a child with severe aplastic anemia. Eltrombopag resulted in recovery of platelet count with no need for platelet transfusion support with no reported side effects...
June 27, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28642072/recombinant-human-thrombopoietin-promotes-platelet-engraftment-and-improves-prognosis-of-patients-with-myelodysplastic-syndromes-or-aplastic-anemia-after-allogeneic-hematopoietic-stem-cell-transplantation
#20
Hong Wang, Man Huang, Ying Zhao, Jia-Qian Qi, Chun Chen, Ya-Qiong Tang, Hui-Ying Qiu, Cheng-Cheng Fu, Xiao-Wen Tang, De-Pei Wu, Chang-Geng Ruan, Yue Han
Poor platelet graft function (PPGF) is a significant complication after allogeneic hematopoietic stem cell transplantation (allo-HSCT). However, no optimal treatment has been recommended. This study investigated aspects of platelet recovery after allo-HSCT, including prognostic value and the effect of recombinant human thrombopoietin (rhTPO). We retrospectively analyzed 275 patients who received allo-HSCT in our center. Of them, 135 (49.1%) patients had good platelet graft function (GPGF) and 140 (50.9%) had PPGF...
June 19, 2017: Biology of Blood and Marrow Transplantation
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