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Aplastic anemia

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https://www.readbyqxmd.com/read/29046759/copper-deficiency-a-new-triad-anemia-leucopenia-and-myeloneuropathy
#1
Shoaib M Wazir, Ibrahim Ghobrial
Clinical copper deficiency is now more frequently recognized. Hematologically, it can present as anemia (microcytic, normocytic, or macrocytic) and neutropenia. Thrombocytopenia is relatively rare. Neurologically, it can manifest as myelopathy and peripheral neuropathy simulating subacute combined degeneration. Bone marrow findings can mimic myelodysplasia resulting in occasional inappropriate referral for bone marrow transplantation. Other conditions with similar presentations include infections, drug toxicity, autoimmunity, B12 deficiency, folate deficiency, myelodysplastic syndrome, aplastic anemia, and lymphoma with bone marrow involvement...
October 2017: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/29046282/clonality-in-context-hematopoietic-clones-in-their-marrow-environment
#2
James N Cooper, Neal S Young
Clonal hematopoiesis occurs normally, especially with aging, and in the setting of disease, not only myeloid cancers but in bone marrow failure. In cancer, malignant clones are characterized by recurrent somatic mutations in specific sets of genes, but the direct relationship of such mutations to leukemogenesis, when they occur in an apparently healthy older individual or after recovery from immune aplastic anemia, is uncertain. Here we emphasize a view of clonal evolution that stresses natural selection over deterministic ontogeny, and we stress the selective role of the marrow's, and organism's, environment...
October 18, 2017: Blood
https://www.readbyqxmd.com/read/29046127/serum-ferritin-is-a-different-predictor-from-transfusion-history-for-allogeneic-transplantation-outcome-in-patients-with-severe-aplastic-anemia
#3
Xiaoyu Zhang, Yuanyuan Shi, Yong Huang, Guixin Zhang, Yi He, Erlie Jiang, Jialin Wei, Donglin Yang, Qiaoling Ma, Rongli Zhang, Weihua Zhai, Sizhou Feng, Mingzhe Han
OBJECTIVES: Severe aplastic anemia (SAA) patients receive more red blood cell (RBC) transfusions as supportive management. We aim to clarify the associations between transfusion history or pre-transplantation serum ferritin (SF) and the overall survival of allogeneic hematopoietic stem cell transplantation (allo-HSCT) among SAA patients. MATERIAL AND METHODS: We retrospectively investigated 96 SAA patients undergoing allo-HSCT, and grouped them according to pre-transplantation duration...
October 18, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/29043236/efficacy-of-eculizumab-in-paroxysmal-nocturnal-hemoglobinuria-patients-with-or-without-aplastic-anemia-prospective-study-of-a-korean-pnh-cohort
#4
Chul Won Choi, Jun Ho Jang, Jin Seok Kim, Deog-Yeon Jo, Je-Hwan Lee, Sung-Hyun Kim, Yeo-Kyeoung Kim, Jong-Ho Won, Joo Seop Chung, Hawk Kim, Jae Hoon Lee, Min Kyoung Kim, Hyeon-Seok Eom, Shin Young Hyun, Jeong-A Kim, Jong Wook Lee
BACKGROUND: Patients with paroxysmal nocturnal hemoglobinuria (PNH) often have concurrent aplastic anemia (AA). This study aimed to determine whether eculizumab-treated patients show clinical benefit regardless of concurrent AA. METHODS: We analyzed 46 PNH patients ≥18 years of age who were diagnosed by flow cytometry and treated with eculizumab for more than 6 months in the prospective Korean PNH registry. Patients were categorized into two groups: PNH patients with concurrent AA (PNH/AA, N=27) and without AA (classic PNH, N=19)...
September 2017: Blood Research
https://www.readbyqxmd.com/read/29037549/long-term-outcome-of-pregnancy-complicating-with-severe-aplastic-anemia-under-supportive-care
#5
Kuan-Ju Chen, Yao-Lung Chang, Horng Chang, Shen-Yuan Su, Hsiu-Huei Peng, Shuenn-Dyh Chang, An-Shine Chao
OBJECTIVES: Pregnancy associated with aplastic anemia (AA) is a rare and heterogeneous disorder. We aimed to identify and evaluate the maternal and pregnant outcomes of pregnancy-associated severe AA treated with supportive care. MATERIALS AND METHODS: A 25-year retrospective study was conducted at in a single center between 1990 and 2014 with pregnancy associated severe AA. In addition, relevant published cases of antenatally diagnosed pregnancy-associated severe AA after 1990 were identified by PubMed...
October 2017: Taiwanese Journal of Obstetrics & Gynecology
https://www.readbyqxmd.com/read/29032585/infection-surveillance-in-pediatric-hematopoietic-stem-cell-transplantation-recipients
#6
Daniela Caldas Teixeira, Lilian Martins Oliveira Diniz, Paulo Henrique Orlandi Mourão, Fabiana Maria Kakehashi, Antonio Vaz de Macedo, Helena Duani, Wanessa Trindade Clemente, Karla Emília de Sá Rodrigues, Roberta Maia de Castro Romanelli
OBJECTIVE: Describe the profile of reported Healthcare-Associated Infections (HAIs) in pediatric patients submitted to hematopoietic stem cell transplantation (HSCT) at a reference center. METHODS: Retrospective cohort of pediatric patients who were submitted to HSCT from 2008 to 2016. The criteria for HAI were based on those established by the National Healthcare Safety Network. Data were collected by active surveillance performed daily by professionals. This study was approved by the Institutional Research Ethics Committee...
October 15, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/29032265/effect-of-antithymocyte-globulin-source-on-outcomes-of-hla-matched-siblings-allogeneic-hematopoietic-stem-cell-transplantation-for-patients-with-severe-aplastic-anemia
#7
Xin Chen, Jialin Wei, Yong Huang, Yi He, Donglin Yang, Rongli Zhang, Erlie Jiang, Qiaoling Ma, Weihua Zhai, Jianfeng Yao, Guixin Zhang, Sizhou Feng, Mingzhe Han
OBJECTIVE: To evaluate efficacy of porcine antithymocyte globulin (ATG) in HLA-matched siblings allogeneic hematopoietic stem cell transplantation (MSD-HSCT) for patients with severe aplastic anemia (SAA). METHODS: The clinical data of 113 SAA patients who received MSD-HSCT from January 2005 to November 2016 were analyzed retrospectively. Of these, 58 patients received rabbit ATG as a part of conditioning regimen (R-ATG group), while the other 55 patients received porcine ATG (P-ATG group)...
October 12, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28988743/fludarabine-based-reduced-intensity-regimen-for-matched-related-donor-hematopoietic-stem-cell-transplantation-in-acquired-severe-aplastic-anemia
#8
K M Ghanem, M A Kharfan-Dabaja, H El-Solh, N Harris, N Kreidieh, R Nehme, S A Muwakkit, R Saab, A Bazarbachi, M R Abboud
Different conditioning regimens have been evaluated in matched-related donor allogeneic hematopoietic stem cell transplantation (allo-HSCT) for acquired severe aplastic anemia (SAA) with varying results. In this manuscript, we report our experience with fludarabine (120mg/m(2)), very low dose cyclophosphamide (1200mg/m(2)) and antithymocyte globulin (7.5mg/kg). Low dose total body irradiation (2Gy) was added to the conditioning regimen for patients older than 15 years. Nineteen patients (median age 23years) underwent transplant between 2008 and 2015...
October 4, 2017: Current Research in Translational Medicine
https://www.readbyqxmd.com/read/28978826/progress-in-iron-metabolism-research
#9
Hiroshi Kawabata
Iron is essential for various cellular processes, but an excess of iron may cause organ damage through the production of reactive oxygen species. Therefore, the amount of iron in the body must be strictly controlled. The central regulator of systemic iron homeostasis is hepcidin, which is primarily produced in the liver. Various molecules, including HFE, transferrin receptor 2 (TFR2), and hemojuvelin (HJV), are involved in sensing systemic iron status. Hepatocytes produce hepcidin in response to excess iron and inflammatory stimuli (e...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28978824/the-cutting-edge-of-aplastic-anemia-treatment
#10
Naoshi Obara
Aplastic anemia is a syndrome in which hematopoietic stem cells are decreased and bone marrow hypoplasia and pancytopenia are observed; it is considered as a T cell-mediated autoimmune disease. Recently, it has been reported that gene mutations suggestive of clonal hematopoiesis are detected in approximately one third of the patients with aplastic anemia. Among treatment approaches other than hematopoietic stem cell transplantation, immunosuppressive therapy with antithymocyte globulin (ATG) plus cyclosporin is a basic approach, although it has been shown that eltrombopag, a thrombopoietin receptor agonist, is effective and that the recovery of hematopoiesis in three blood lineage is observed in some patients...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28971166/somatic-hla-mutations-expose-the-role-of-class-i-mediated-autoimmunity-in-aplastic-anemia-and-its-clonal-complications
#11
Daria V Babushok, Jamie L Duke, Hongbo M Xie, Natasha Stanley, Jamie Atienza, Nieves Perdigones, Peter Nicholas, Deborah Ferriola, Yimei Li, Hugh Huang, Wenda Ye, Jennifer J D Morrissette, Jane Kearns, David L Porter, Gregory M Podsakoff, Laurence C Eisenlohr, Jaclyn A Biegel, Stella T Chou, Dimitrios S Monos, Monica Bessler, Timothy S Olson
Acquired aplastic anemia (aAA) is an acquired deficiency of early hematopoietic cells, characterized by inadequate blood production, and a predisposition to myelodysplastic syndrome (MDS) and leukemia. Although its exact pathogenesis is unknown, aAA is thought to be driven by Human Leukocyte Antigen (HLA)-restricted T cell immunity, with earlier studies favoring HLA class II-mediated pathways. Using whole exome sequencing (WES), we recently identified two aAA patients with somatic mutations in HLA class I genes...
October 10, 2017: Blood Advances
https://www.readbyqxmd.com/read/28970690/successful-surgical-procedure-in-a-patient-of-aplastic-anemia-with-platelet-refractoriness-using-cross-match-compatible-platelets
#12
Ritam Chakrabarty, Sudipta Sekhar Das
This case marks the beginning of issuing cross-matched platelet products in Eastern India. A known case of aplastic anemia, on regular transfusion support, now presented with obstructed ventral periumbilical hernia requiring urgent surgical intervention. Platelet count at presentation was 13,000/μL. Platelet cross-matching was done by solid phase method. Ten units of random donor platelets were crossmatched. Five units were compatible and transfused. Counts rose to 84,000/μL after 1 h. Surgery was completed successfully...
July 2017: Asian Journal of Transfusion Science
https://www.readbyqxmd.com/read/28970677/red-cell-alloimmunization-in-repeatedly-transfused-patients
#13
Dimel K Bhuva, Jitendra H Vachhani
INTRODUCTION: Repeated blood transfusions can result in the production of alloantibodies against one or more red cell antigens, which complicates subsequent transfusions. Aims: The study was done to find incidence of various red cell alloantibodies; to determine the type of alloantibody; to identify the factors such as frequency of transfusion, splenectomy status, donor ethnicity and gender and their association with the development of antibody in repeatedly transfused patients. MATERIALS AND METHODS: This study was carried out in Dept...
July 2017: Asian Journal of Transfusion Science
https://www.readbyqxmd.com/read/28968996/apc-c-is-essential-for-hematopoiesis-and-impaired-in-aplastic-anemia
#14
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...
September 8, 2017: Oncotarget
https://www.readbyqxmd.com/read/28967434/-sarcoid-like-granulomatosis-associated-with-eosinophilic-fasciitis
#15
E Zuelgaray, C Sallé de Chou, M-D Vignon-Pennamen, M Battistella, S Leonard-Louis, L Hefez, F Guibal, M Bagot, J-D Bouaziz
BACKGROUND: Eosinophilic fasciitis (EF) is a rare condition characterized by swelling of the extremities, sclerodermatous evolution and frequent hypereosinophilia. Hematological disorders, including aplastic anemia, solid tumors and autoimmune diseases, may be associated with EF. EF is usually not associated with granulomatous diseases. CASE REPORT: Herein we describe the case of an 80-year-old man with symmetrical swelling and sclerosis of the legs, typical of EF, associated with skin and lymph node granulomas...
September 26, 2017: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/28965225/horse-versus-rabbit-antithymocyte-globulin-in-immunosuppressive-therapy-of-treatment-na%C3%A3-ve-aplastic-anemia-a-systematic-review-and-meta-analysis
#16
Nan Yang, Jinqiu Chen, Hui Zhang, Zhiming Dai, Huan Yao, Xiaorong Ma, Ju Bai, Yilin Zhang, Wanggang Zhang
The first-line formulation of antithymocyte globulin (ATG) remains unknown. We aimed to systematically review evidence to compare the efficacy and safety profiles of different ATGs. We did a systematic review and meta-analysis of randomized controlled trials (RCTs) and cohort controlled studies comparing horse and rabbit ATG in immunosuppressive therapy of treatment-naïve aplastic anemia. We searched The Cochrane Library, PubMed, EMBASE, ClinicalTrials.gov , and conference proceedings of American Society of Hematology and European Society for Blood and Marrow Transplantation annual meetings...
September 30, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28960909/allogeneic-bone-marrow-derived-mesenchymal-stromal-cells-expanded-in-vitro-for-treatment-of-aplastic-anemia-a-multicenter-phase-ii-trial
#17
Yan Pang, Hao-Wen Xiao, Hang Zhang, Zeng-Hui Liu, Li Li, Yang Gao, Hong-Bo Li, Zu-Jun Jiang, Huo Tan, Jing-Ren Lin, Xin Du, Jian-Yu Weng, Da-Nian Nie, Dong-Jun Lin, Xiang-Zhong Zhang, Qi-Fa Liu, Duo-Rong Xu, Hai-Jia Chen, Xiao-Hu Ge, Xiao-Yan Wang, Yang Xiao
No abstract text is available yet for this article.
October 2017: Stem Cells Translational Medicine
https://www.readbyqxmd.com/read/28954343/-improved-outcome-by-optimized-conditioning-regimens-with-an-increased-dose-of-cyclophosphamide-in-allogeneic-peripheral-blood-stem-cell-transplantation-for-severe-aplastic-anemia
#18
Y Huang, Y He, X Liu, D L Yang, R L Zhang, E L Jiang, Q L Ma, J L Wei, S Z Feng, M Z Han
Objective: To respectively analyze the impact of conditioning regimens with a dose-decreased cyclophosphamide (Cy) on the outcome in fully matched sibling donor (MSD) peripheral blood stem cell transplantation (PBSCT) for severe aplastic anemia (SAA) . Methods: Two conditioning regimens with different doses of Cy (150 mg/kg or 120 mg/kg) in combination with fludarabine (Flu) and antithymocyte globulin (ATG) for MSD-PBSCT were investigated in 51 patients with acquired SAA. Results: Overall survival and failure-free survival in patients received 150 mg/kg of Cy (Cy150 cohort) or 120 mg/kg (Cy120 cohort) were 93...
August 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28951880/chinese-herbal-formula-modified-danggui-buxue-tang-attenuates-apoptosis-of-hematopoietic-stem-cells-in-immune-mediated-aplastic-anemia-mouse-model
#19
Jingwei Zhou, Xue Li, Peiying Deng, Yi Wei, Juan Liu, Meng Chen, Yamei Xu, Dongmei Zhang, Lingqun Zhu, Lixia Lou, Bin Dong, Qiushuo Jin, Limin Chai
A derivative formula, DGBX, which is composed of three herbs (Radix astragali, Radix Angelicae sinensis, and Coptis chinensis Franch), is derived from a famous Chinese herbal formula, Danggui Buxue Tang (DBT) (Radix astragali and Radix Angelicae sinensis). We aimed to investigate the effects of DGBX on the regulation of the balance between proliferation and apoptosis of hematopoietic stem cells (HSCs) due to the aberrant immune response in a mouse model of aplastic anemia (AA). Cyclosporine (CsA), an immunosuppressor, was used as the positive control...
2017: Journal of Immunology Research
https://www.readbyqxmd.com/read/28951259/chimerism-in-pediatric-hematopoietic-stem-cell-transplantation-and-its-correlation-with-the-clinical-outcome
#20
Hala Gabr, Ilham Youssry, Yasmin El-Ansary, Ghada Mosallam, Nermine Magdi Riad, Mariam Onsy F Hanna
Hematopoietic stem cell transplantation (HSCT) is the only hope to cure many inherited and acquired hematological disorders in children. Monitoring of chimerism helps to predict the post-transplantation events, with the intention to enhance the long-term disease free survival (DFS). The study aimed to investigate the importance of early chimerism detection to predict the clinical outcome following HSCT. The study included nine recipients (six β-thalassemia and three severe aplastic anemia patients) and their 10/10 HLA identical sibling donors...
September 23, 2017: Transplant Immunology
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