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Severe aplastic anemia

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https://www.readbyqxmd.com/read/29224329/-allogeneic-hematopoietic-stem-cell-transplantation-for-the-treatment-of-severe-aplastic-anemia-complicated-with-hepatocellular-carcinoma-a-case-report-and-literature-review
#1
S W Yang, Z M Zhu, X L Yuan, L Jiang, R J Ma, Y Zhang
No abstract text is available yet for this article.
November 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/29222240/old-and-new-tools-in-the-clinical-diagnosis-of-inherited-bone-marrow-failure-syndromes
#2
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/29203412/comparable-outcomes-after-hla-matched-sibling-and-alternative-donor-hematopoietic-cell-transplantation-for-children-with-fanconi-anemia-and-severe-aplastic-anemia
#3
Christen L Ebens, Todd E DeFor, Rebecca Tryon, John E Wagner, Margaret L MacMillan
Fanconi anemia (FA) associated severe aplastic anemia (SAA) requires allogeneic hematopoietic cell transplantation (HCT) for cure. With the evolution of conditioning regimens over time, alternative donor HCT (AD-HCT) outcomes have dramatically improved. We compared outcomes of HLA-matched sibling donor HCT (MSD-HCT, n=17) to AD-HCT (n=57) for FAassociated SAA at a single institution from 2001-2016. Overall survival at five years was 94 vs. 86%, neutrophil engraftment 100 vs. 95%, platelet recovery 100 vs. 89%, grade II-IV acute GVHD 6 vs...
December 1, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29173808/epigenetic-and-microenvironmental-alterations-in-bone-marrow-associated-with-ros-in-experimental-aplastic-anemia
#4
Ritam Chatterjee, Sujata Law
Aplastic anemia or bone marrow failure often develops as an effect of chemotherapeutic drug application for the treatment of various pathophysiological conditions including cancer. The long-term bone marrow injury affects the basic hematopoietic population including hematopoietic stem/progenitor cells (HSPCs). The present study aimed in unearthing the underlying mechanisms of chemotherapeutics mediated bone marrow aplasia with special focus on altered redox status and associated effects on hematopoietic microenvironment and epigenetic status of hematopoietic cells...
November 22, 2017: European Journal of Cell Biology
https://www.readbyqxmd.com/read/29170252/nationwide-survey-on-the-use-of-eltrombopag-in-patients-with-severe-aplastic-anemia-a-report-on-behalf-of-the-french-reference-center-for-aplastic-anemia
#5
Etienne Lengline, Bernard Drenou, Pierre Peterlin, Olivier Tournilhac, Julie Abraham, Ana Berceanu, Brigitte Dupriez, Gaelle Guillerm, Emmanuel Raffoux, Flore Sicre de Fontbrune, Lionel Ades, Marie Balsat, Driss Chaoui, Paul Coppo, Selim Corm, Thierry Leblanc, Natacha Maillard, Louis Terriou, Gerard Socié, Regis Peffault de la Tour
Few therapeutic options are available for aplastic anemia patients ineligible for transplantation or refractory to immunosuppressive therapy. Eltrombopag recently showed tri-lineage responses in refractory patients. However, real-life use of this drug remains unknown. This retrospective study (2012-2016) was conducted by the French Reference Center for Aplastic anemia on patients with relapse/refractory aplastic anemia, and patients ineligible for anti-thymocyte globulin (naive) or transplantation, who received eltrombopag for at least two months...
November 23, 2017: Haematologica
https://www.readbyqxmd.com/read/29146883/a-landscape-of-germline-mutations-in-a-cohort-of-inherited-bone-marrow-failure-patients
#6
Olivier Bluteau, Marie Sebert, Thierry Leblanc, Régis Peffault de Latour, Samuel Quentin, Elodie Lainey, Lucie Hernandez, Jean-Hugues Dalle, Flore Sicre de Fontbrune, Etienne Lengline, Raphael Itzykson, Emmanuelle Clappier, Nicolas Boissel, Naddia Vasquez, Mélanie Da Costa, Julien Masliah-Planchon, Wendy Cuccuini, Anna Raimbault, Louis De Jaegere, Lionel Adès, Pierre Fenaux, Sébastien Maury, Claudine Schmitt, Marc Muller, Carine Domenech, Nicolas Blin, Bénédicte Bruno, Isabelle Pellier, Mathilde Hunault, Stéphane Blanche, Arnaud Petit, Guy Leverger, Gérard Michel, Yves Bertrand, André Baruchel, Gérard Socié, Jean Soulier
Bone marrow failure (BMF) in children and young adults is often suspected to be inherited, but in many cases diagnosis remains uncertain. We studied a cohort of 179 patients (from 173 families) with BMF of suspected inherited origin but unresolved diagnosis after medical evaluation and Fanconi anemia exclusion. All patients had cytopenias, and 12.0% presented ≥5% bone marrow blast cells. Median age at genetic evaluation was 11 years; 20.7% of patients were aged ≤2 years and 36.9% were ≥18 years. We analyzed genomic DNA from skin fibroblasts using whole-exome sequencing, and were able to assign a causal or likely causal germline mutation in 86 patients (48...
November 16, 2017: Blood
https://www.readbyqxmd.com/read/29143887/diagnosis-and-treatment-of-aplastic-anemia
#7
REVIEW
Scott A Peslak, Timothy Olson, Daria V Babushok
Acquired aplastic anemia (AA) is a rare, life-threatening bone marrow failure (BMF) disorder that affects patients of all ages and is caused by lymphocyte destruction of early hematopoietic cells. Diagnosis of AA requires a comprehensive approach with prompt evaluation for inherited and secondary causes of bone marrow aplasia, while providing aggressive supportive care. The choice of frontline therapy is determined by a number of factors including AA severity, age of the patient, donor availability, and access to optimal therapies...
November 16, 2017: Current Treatment Options in Oncology
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
#8
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
https://www.readbyqxmd.com/read/29137385/improved-outcome-of-haploidentical-transplantation-in-severe-aplastic-anemia-using-reduced-intensity-fludarabine-based-conditioning
#9
Wu Yamei, Luo Rongmu, Cao Yongbin, Si Yingjian, Li Xiaohong, Zhang Xiaomei, Yan Pei, Du Zhenlan, Wang Haitao, Wang Jing, Wang Bojing, Wu Xiaoxiong, Da Wanming
Significant improvements in hematopoietic stem cell transplantation (HSCT) with haploidentical family donors (HFD) have confirmed its therapeutic role in severe aplastic anemia (SAA) and led to the evolution of treatment algorithms. However, the optimal conditioning regimen for HFD-HSCT remains undefined, especially the dosage of cyclophosphamide (Cy). A total of 77 patients with SAA from two research centers, who received HFD-HSCT with reduced-intensity fludarabine + cyclophosphamide + thymoglobulin ± busulfan conditioning regimen plus third-party cells infusion were included in this study, of which 67 pairs had 4-5 loci mismatched...
October 13, 2017: Oncotarget
https://www.readbyqxmd.com/read/29127946/seasonal-trends-of-diagnosis-of-childhood-malignant-diseases-and-viral-prevalence-in-south-korea
#10
Kyu Seok Shim, Min Hyung Kim, Choong Nam Shim, Minkyu Han, In Seok Lim, Soo Ahn Chae, Sin Weon Yun, Na Mi Lee, Dae Yong Yi, Hyery Kim
BACKGROUND: Several studies have reported a seasonal trend in the diagnosis of childhood cancer suggesting seasonal factors such as infection. The present study aimed to analyze the diagnosis pattern of childhood malignant diseases using public health data, and to compare this pattern with seasonal viral infection trends. METHOD: Using the open data source of the Health Insurance Review and Assessment Service, we extracted data regarding all patients under 21 years of age and who had any cancer, aplastic anemia or myelodysplastic syndrome between September 2009 and December 2013...
November 8, 2017: Cancer Epidemiology
https://www.readbyqxmd.com/read/29125443/neurosurgical-management-in-children-with-bleeding-diathesis-auditing-neurological-outcome
#11
Zaitun Zakaria, Chandrasekaran Kaliaperumal, Darach Crimmins, John Caird
OBJECTIVE The aim of this study was to assess the outcome of neurosurgical treatment in children with bleeding diathesis and also to evaluate the current management plan applied in the authors' service. METHODS The authors retrospectively analyzed all cases in which neurosurgical procedures were performed in pediatric patients presenting with intracranial hematoma due to an underlying bleeding tendency over a 5-year period at their institution. They evaluated the patients' neurological symptoms from the initial referral, hematological abnormalities, surgical treatment, neurological outcome, and scores on the Pediatric Glasgow Outcome Scale-Extended (GOS-E Peds) obtained 1 year after the last operation...
November 10, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/29119669/sinusitis-caused-by-exserohilum-rostratum-after-cord-blood-transplantation-for-myelodysplastic-syndrome-a-case-report-and-literature-review
#12
Sumiko Kohashi, Takaaki Toyama, Norisato Hashimoto, Masatoshi Sakurai, Jun Kato, Taku Kikuchi, Yuya Koda, Kayoko Sugita, Naoki Hasegawa, Kyoko Yarita, Katsuhiko Kamei, Shinichiro Okamoto, Takehiko Mori
Invasive fungal disease is a serious infectious complication of allogeneic hematopoietic stem cell transplantation (HSCT). Exserohilum rostratum is a species causing phaeohyphomycosis, which rarely causes invasive disease in humans. We treated a case of sinusitis caused by E. rostratum after cord blood transplantation (CBT). A 60-year-old man with myelodysplastic syndrome, who had a medical history of an operation to correct deviation of the nasal septum, developed sinusitis caused by E. rostratum under prolonged profound neutropenia after a second CBT because of the graft rejection of the first transplantation...
November 8, 2017: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/29115638/the-shortening-telomere-length-of-t-lymphocytes-maybe-associated-with-hyper%C3%A2-function-in-servere-aplastic-anemia
#13
Chaomeng Wang, Tian Zhang, Yihao Wang, Yang Li, Chunyan Liu, Hui Liu, Lijuan Li, Kai Ding, Ting Wang, Honglei Wang, Zonghong Shao, Rong Fu
Severe aplastic anemia (SAA) is a primary disorder of severe bone marrow failure characterizing with extreme pancytopenia and a profound diminution of bone marrow progenitor cells, which is associated with T cell hyper‑function. Abnormal telomere shortening of bone marrow mononuclear cell has been reported in AA, which may lead to genomic instability, and result in cell senescence or apoptosis. Notably, certain studies identfieid that lymphocytes of shortening telomere length have undergone apoptosis escape in autoimmune diseases...
November 8, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29100905/post-transplant-cyclophosphamide-ptcy-as-sole-gvhd-prophylaxis-is-feasible-in-patients-undergoing-peripheral-blood-stem-cell-transplantation-for-severe-aplastic-anaemia-using-matched-sibling-donors
#14
Biju George, Nisham Pn, Anup J Devasia, Uday Kulkarni, Anu Korula, Kavitha M Lakshmi, Aby Abraham, Alok Srivastava, Vikram Mathews
High-dose cyclophosphamide (PTCY) following allogeneic hematopoietic cell transplantation (HSCT) has been shown to be effective in preventing graft-versus-host disease (GVHD) following HLA-matched bone marrow transplantation. We performed a phase II study of PTCY given at 50 mg/kg IV on days 3 and 4 as sole GVHD prophylaxis following HSCT for severe aplastic anemia (SAA) receiving G-CSF-mobilized peripheral blood stem cell (PBSC) grafts from HLA-matched related donors following conditioning with fludarabine, cyclophosphamide and single dose Total Body Irradiation (TBI)...
October 31, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29094192/changes-in-bi-ventricular-function-after-hematopoietic-stem-cell-transplant-as-assessed-by-speckle-tracking-echocardiography
#15
Stuart Covi, Yaddanapudi Ravindranath, Ahmad Farooqi, Sureyya Savasan, Roland Chu, Sanjeev Aggarwal
Hematopoietic stem cell transplant (HSCT) is a therapeutic option for patients with sickle cell disease (SCD) and severe acquired aplastic anemia (SAA). HSCT may have beneficial effects on ventricular function in damaged myocardium. We hypothesized improvement in ventricular performance and pulmonary hypertension following HSCT with strain echocardiography in SCD and SAA. Echocardiographic strain and other standard functional data were obtained via retrospective cohort analysis of patients (n = 23) with SCD and SAA who underwent HSCT and were followed at a single center between 2000 and 2014...
November 1, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/29081205/-advances-on-thrombopoietin-receptor-agonist-in-severe-aplastic-anemia
#16
J Du, F K Zhang
No abstract text is available yet for this article.
September 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/29081192/-risk-factor-analysis-of-poor-graft-function-after-allogeneic-hematopoietic-stem-cell-transplantation-for-severe-aplastic-anemia
#17
C Y Shi, Z H Mamal, X X Liu, L H Wu, D N Xia, Y R Nie, F Q Lai, H W Duan, Z J Xiao, Y H Jiang, Y Li, Yang Xiao
Objective: To investigate the risk factors of poor graft function (PGF) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) for severe aplastic anemia (SAA) . Methods: Clinical data from 111 SAA patients who received allo-HSCT were analyzed retrospectively. Factors including age, gender, interval to transplantation, the level of serum ferritin before transplantation were analyzed by Cox multivariate regression analysis. Results: Among the 111 patients who underwent allo-HSCT, 16 developed PGF (14...
September 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/29079125/hereditary-persistence-of-hemoglobin-f-is-protective-against-red-cell-sickling-a-case-report-and-brief-review
#18
Alexandra Sokolova, Anton Mararenko, Alexander Rozin, Alida Podrumar, Vladimir Gotlieb
Fetal hemoglobin (HbF) is a physiologic protein tetramer that is crucial for a developing fetus to survive in utero. Maternal hemoglobin has a relatively lower affinity for oxygen, and thus allows for an efficient transfer of oxygen from maternal to fetal blood. In addition to fulfilling a critical physiologic role, HbF is also known to alleviate symptoms of sickle-cell disease (SCD). The concentration of HbF depends on several factors. HbF is elevated in inherited conditions, such as hereditary persistence of HbF, hereditary spherocytosis, and thalassemia...
October 16, 2017: Hematology/oncology and Stem Cell Therapy
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
#19
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/29037549/long-term-outcome-of-pregnancy-complicating-with-severe-aplastic-anemia-under-supportive-care
#20
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
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