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https://www.readbyqxmd.com/read/29146883/a-landscape-of-germline-mutations-in-a-cohort-of-inherited-bone-marrow-failure-patients
#1
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
#2
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
#3
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
#4
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/29131151/combined-intensive-immunosuppression-and-eculizumab-for-aplastic-anemia-in-the-context-of-hemolytic-paroxysmal-nocturnal-hemoglobinuria-a-retrospective-analysis
#5
S Pagliuca, A M Risitano, F S De Fontbrune, M Robin, A P Iori, S Marotta, D Michonneau, A Villate, D Desmier, G Socié, R P De Latour
No abstract text is available yet for this article.
November 13, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/29128072/paroxysmal-nocturnal-hemoglobinuria-assessment-by-flow-cytometric-analysis
#6
REVIEW
Mike Keeney, Andrea Illingworth, D Robert Sutherland
Paroxysmal nocturnal hemoglobinuria (PNH) is an uncommon but frequently debilitating disease that, if untreated, may lead to death in up to 35% of patients within 5 years. Assessment of PNH clone size by flow cytometric analysis has increased in importance with the availability of therapeutic treatments, which prevent the hemolysis of red blood cells and, hence, the myriad symptoms that accompany the disease. This article addresses flow cytometric methodologies and highlights areas of importance in implementing testing, not only for classic PNH but also for other related bone marrow failure disorders, such as aplastic anemia and low-grade myelodysplastic syndrome...
December 2017: Clinics in Laboratory Medicine
https://www.readbyqxmd.com/read/29127946/seasonal-trends-of-diagnosis-of-childhood-malignant-diseases-and-viral-prevalence-in-south-korea
#7
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
#8
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
#9
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
#10
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/29108725/aplastic-anemia-in-two-consecutive-pregnancies-obstetric-and-anesthetic-management
#11
E Riveros-Perez, A Hermesch, L Barbour, J Hawkins
Aplastic anemia is a serious condition occasionally coexisting with pregnancy. This pathological process is associated with significant maternal and neonatal morbidity and mortality. Obstetric and anesthetic management are particularly challenging, and treatment requires knowledge of pathophysiologic mechanisms in order to provide safe care to this group of patients. We describe the successful obstetric management and labor analgesia of a patient with a diagnosis of aplastic anemia in two consecutive pregnancies...
September 14, 2017: International Journal of Obstetric Anesthesia
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
#12
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
#13
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
#14
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
#15
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
#16
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/29075064/role-of-arsenic-trioxide-in-the-management-of-aplastic-anemia
#17
Gaurav Prakash, Uday Yanamandra, Alka Khadwal, Neelam Varma, Subhash Varma, Pankaj Malhotra
Aplastic anemia is a common problem in the developing world with therapeutic challenges as most of the patients are non-affording to standard care owing to resource constraints. We present the results of an open label single arm, non-randomized, single center, prospective phase II trial of the compassionate use of arsenic trioxide in patients refractory to ATG or unable to afford the standard of care therapy. The study was prematurely terminated at eighth week due to non-response in 100% of patients and death in two patients...
December 2017: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/29070131/-efficacy-of-shengxue-mixture-combined-with-intraosseous-infusion-for-treatment-of-aplastic-anemia
#18
Shan-Xi Liu, Yi-Guo Liu, Peng-Hui Wang, Mao-Feng Qi, Meng Luo
OBJECTIVE: To evaluate the efficacy and safety of Shengxue mixture combined with intraosseous blood infusion for treatment of aplastic anemia patients. METHODS: From 2011 to 2015, Institute of blood diseases of Shaanxi Medical University admitted 53 patients with aplastic anemia. The patients were treated with shengxue mixture 200 ml, orally, twice a day. Stanozolol tablets, Adult 2 mg, three times a day, mycophenolate mofetil 1.0 g, twice a day. Intraosseous infusion of the following medicine were administered in patients: recombinant human EPO 10000 U, recombinant human G-CSF 450 µg, recombinant human IL-11 4...
October 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/29068868/elevated-serum-interleukin-6-predicts-favorable-response-to-immunosuppressive-therapy-in-children-with-aplastic-anemia
#19
Shuanglong Lu, Xiaohong Qiao, Xiaotian Xie
BACKGROUND: Immunosuppressive therapy (IST) is the standard treatment for aplastic anemia (AA) children who lack a sibling donor, but the clinical response rate to IST varies. Predictors of response to IST are valuable for stratifying AA patients and making clinical decisions. METHODS: The serum interleukin (IL)-6 levels of 41 AA patients were measured at the time of diagnosis and the response rate of the patients to IST was evaluated at 3, 6, and 12 months after IST...
November 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29046759/copper-deficiency-a-new-triad-anemia-leucopenia-and-myeloneuropathy
#20
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
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