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https://www.readbyqxmd.com/read/28332891/the-positive-effect-of-deferasirox-on-erythropoiesis-in-a-patient-with-a-dual-diagnosis-of-myelofibrosis-and-diffuse-large-b-cell-lymphoma
#1
Louise Kostos, Khai Li Chai, Constantine S Tam, Ali Bazargan
No abstract text is available yet for this article.
March 23, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28316434/comparison-of-deferasirox-and-deferoxamine-effects-on-iron-overload-and-immunological-changes-in-patients-with-blood-transfusion-dependent-%C3%AE-thalassemia
#2
Hayder M Al-Kuraishy, Ali I Al-Gareeb
INTRODUCTION: Beta-thalassemias are a cluster of inherited (autosomal recessive) hematological disorders prevalent in the Mediterranean area due to defects in synthesis of β chains of hemoglobin. The aim of present study was to compare the effects of deferasirox and deferoxamine on iron overload and immunological changes in patients with blood transfusion-dependent β-thalassemia major and intermedia. PATIENTS AND METHODS: This study involved 64 patients with known cases of β-thalassemia major or intermedia that has been treated with blood transfusion and iron chelators...
January 2017: Asian Journal of Transfusion Science
https://www.readbyqxmd.com/read/28296163/long-term-safety-and-efficacy-of-deferasirox-in-young-pediatric-patients-with-transfusional-hemosiderosis-results-from-a-5-year-observational-study-entrust
#3
Elliott Vichinsky, Amal El-Beshlawy, Azzam Al Zoebie, Annie Kamdem, Suzanne Koussa, Thirachit Chotsampancharoen, Andreas Bruederle, Geralyn Gilotti, Jackie Han, Mohsen Elalfy
BACKGROUND: Children with red blood cell disorders may receive regular transfusions from an early age and consequently accumulate iron. Adequate iron chelation therapy can prevent organ damage and delayed growth/development. Deferasirox is indicated for treatment of pediatric patients with chronic iron overload due to transfusional hemosiderosis; however, fewer than 10% of patients in the registration studies were aged 2 to less than 6 years. PROCEDURE: Deferasirox, a once-daily oral iron chelator, was evaluated in young pediatric patients with transfusional hemosiderosis during the observational 5-year ENTRUST study...
March 10, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28287621/restoring-the-impaired-cardiac-calcium-homeostasis-and-cardiac-function-in-iron-overload-rats-by-the-combined-deferiprone-and-n-acetyl-cysteine
#4
Suwakon Wongjaikam, Sirinart Kumfu, Juthamas Khamseekaew, Siriporn C Chattipakorn, Nipon Chattipakorn
Intracellular calcium [Ca(2+)]i dysregulation plays an important role in the pathophysiology of iron overload cardiomyopathy. Although either iron chelators or antioxidants provide cardioprotection, a comparison of the efficacy of deferoxamine (DFO), deferiprone (DFP), deferasirox (DFX), N-acetyl cysteine (NAC) or a combination of DFP plus NAC on cardiac [Ca(2+)]i homeostasis in chronic iron overload has never been investigated. Male Wistar rats were fed with either a normal diet or a high iron (HFe) diet for 4 months...
March 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28248443/iron-overload-induces-apoptosis-of-murine-preosteoblast-cells-via-ros-and-inhibition-of-akt-pathway
#5
Jun-Yu Ke, Wan-Jing Cen, Xue-Zhong Zhou, Ye-Rong Li, Wei-Dong Kong, Jian-Wei Jiang
OBJECTIVE: This study investigates the inhibitory effect of iron overload on MC3T3-E1 cells and its molecular mechanism. METHODS: MC3T3-E1 cells were grown under different concentrations of FAC (ferric ammonium citrate), the WST-8 assay was used to investigate the proliferation of cells following FAC with or without deferasirox. DCFH-DA fluorescence probe was applied to detect the cellular reactive oxygen species (ROS) level. The apoptotic cells were analyzed with Annexin V-FITC/PI and the Hoechst 33258 nuclear staining assay...
March 1, 2017: Oral Diseases
https://www.readbyqxmd.com/read/28243431/combination-iron-chelation-therapy-with-deferiprone-and-deferasirox-in-iron-overloaded-patients-with-transfusion-dependent-%C3%AE-thalassemia-major
#6
Hossein Karami, Mehrnoush Kosaryan, Arash Hadian Amree, Hadi Darvishi-Khezri, Masoomeh Mousavi
There are few papers on the combination therapy of deferiprone (DFP) and deferasirox (DFX) in iron-overloaded patients with transfusion-dependent β-thalassemia major (β-TM). A total of 6 patients with β-TM (5 males and 1 female) with a mean age of 23.8±5.8 years (ranging from 17 to 31) used this treatment regimen. The mean doses of DFP and DFX were 53.9±22.2 and 29.3±6.8 mg/kg/day, respectively. The duration of treatment was 11.5±4.6 months. Their serum ferritin levels were measured to be 2800±1900 and 3400±1600 ng/mL before and after treatment, respectively (p<0...
January 11, 2017: Clinics and Practice
https://www.readbyqxmd.com/read/28221265/acute-liver-failure-during-deferasirox-chelation-a-toxicity-worth-considering
#7
Nathan Menaker, Katharine Halligan, Natasha Shur, John Paige, Matthew Hickling, Anne Nepo, Lauren Weintraub
This case report details a unique case of acute, reversible liver failure in a 12-year-old male with sickle cell anemia on chronic transfusion protocol and deferasirox chelation. There is substantial literature documenting deferasirox-induced renal injury, including Fanconi syndrome, but less documentation of hepatic toxicity and few reports of hepatic failure. The case highlights the importance of close monitoring of ferritin, bilirubin, and transaminases for patients on deferasirox.
April 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28221264/efficacy-and-safety-of-combined-oral-chelation-with-deferiprone-and-deferasirox-in-children-with-%C3%AE-thalassemia-major-an-experience-from-north-india
#8
Nupur Parakh, Jagdish Chandra, Sunita Sharma, Bhavna Dhingra, Rajesh Jain, DeoNath Mahto
OBJECTIVE: A combination of desferrioxamine with either deferiprone (DFP) or deferasirox (DFX) for patients with β-thalassemia major who do not achieve negative iron balance with monotherapy has been studied widely. However, poor compliance resulting from the need for parentral administration of desferrioxamine and its cost necessicitates combining 2 oral chelators. METHODS: A prospective study was conducted in patients with transfusion-dependent β-thalassemia major in a tertiary care center over 2 years...
April 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28185140/cost-utility-analysis-of-three-iron-chelators-used-in-monotherapy-for-the-treatment-of-chronic-iron-overload-in-%C3%AE-thalassaemia-major-patients-an-italian-perspective
#9
Alessia Pepe, Giuseppe Rossi, Anthony Bentley, Maria Caterina Putti, Ludovica Frizziero, Domenico Giuseppe D'Ascola, Liana Cuccia, Anna Spasiano, Aldo Filosa, Vincenzo Caruso, Aishah Hanif, Antonella Meloni
PURPOSE: Deferiprone (DFP), deferasirox (DFX) and deferoxamine (DFO) are used in thalassaemia major (TM) patients to treat chronic iron overload. We evaluated the cost-effectiveness of DFP, compared with DFX and DFO monotherapy, from an Italian healthcare system perspective. METHODS: A Markov model was used over a time horizon of 5 years. Italian-specific cost data were combined with Italian efficacy data. Costs and quality-adjusted life years (QALYs) were calculated for each treatment, with cost-effectiveness expressed as cost per QALY...
February 10, 2017: Clinical Drug Investigation
https://www.readbyqxmd.com/read/28142202/new-film-coated-tablet-formulation-of-deferasirox-is-well-tolerated-in-patients-with-thalassemia-or-lower-risk-mds-results-of-the-randomized-phase-ii-eclipse-study
#10
Ali T Taher, Raffaella Origa, Silverio Perrotta, Alexandra Kourakli, Giovan Battista Ruffo, Antonis Kattamis, Ai-Sim Goh, Annelore Cortoos, Vicky Huang, Marine Weill, Raquel Merino Herranz, John B Porter
Once-daily deferasirox dispersible tablets (DT) have a well-defined safety and efficacy profile and, compared with parenteral deferoxamine, provide greater patient adherence, satisfaction, and quality of life. However, barriers still exist to optimal adherence, including gastrointestinal tolerability and palatability, leading to development of a new film-coated tablet (FCT) formulation that can be swallowed with a light meal, without the need to disperse into a suspension prior to consumption. The randomized, open-label, phase II ECLIPSE study evaluated the safety of deferasirox DT and FCT formulations over 24 weeks in chelation-naïve or pre-treated patients aged ≥10 years, with transfusion-dependent thalassemia or IPSS-R very-low-, low-, or intermediate-risk myelodysplastic syndromes...
January 31, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28141745/deferasirox-iron-complex-formation-ratio-as-an-indicator-of-long-term-chelation-efficacy-in-%C3%AE-thalassemia-major
#11
Meng-Yao Lu, Ting-Hao Lin, Po-Hung Chiang, Pei-Hsin Kuo, Ning Wang, Wen-Hsin Wu, Kai-Hsin Lin, Tzu-Hua Wu
BACKGROUND: β-Thalassemia major patients with higher total drug levels [deferasirox (DEFR) plus its iron complex] do not yield better serum ferritin (SF) control. This study aimed to determine the concentrations of DEFR and its iron complex (Fe-[DEFR]2) in thalassemia patients to predict the chelation efficacy in terms of SF and cardiac T2* values. METHODS: Patients' steady-state drug levels at trough (Ctrough) and 2 hours postdose (C2h) were determined. Because iron deposition may cause changes in the hepatic metabolism of amino acids, the concentrations of 40 amino acids in plasma were also assayed at 2 hours postdose...
April 2017: Therapeutic Drug Monitoring
https://www.readbyqxmd.com/read/28140713/deferasirox-has-strong-anti-leukemia-activity-but-may-antagonize-theanti-leukemia-effect-of-doxorubicin
#12
Yu-Chien Chang, Wen-Jyi Lo, Yu-Ting Huang, Chaio-Lin Lin, Chiu-Che Feng, Hsin-Ting Lin, Hsu-Chen Cheng, Su-Peng Yeh
Deferasirox (DFX), in addition to its iron-chelation property, has marked anti-proliferative effects on cancer cells. However, the activity and mechanism by which DFX inhibits acute myeloid leukemia (AML) cells remain to be elucidated. Furthermore, the anti-leukemia effect of combining DFX with currently recommended agents doxorubicin (DOX) and cytosine arabinoside (Ara-C) has not been studied. In this study, we show that DFX significantly reduces the viability of three AML cell lines, HL60, THP1, and WEHI3 and two primary leukemic cells harvested from AML patients...
January 31, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28139717/deferasirox-induced-iron-depletion-promotes-bclxl-downregulation-and-death-of-proximal-tubular-cells
#13
Diego Martin-Sanchez, Angel Gallegos-Villalobos, Miguel Fontecha-Barriuso, Susana Carrasco, Maria Dolores Sanchez-Niño, Francisco J Lopez-Hernandez, Marta Ruiz-Ortega, Jesus Egido, Alberto Ortiz, Ana Belén Sanz
Iron deficiency has been associated with kidney injury. Deferasirox is an oral iron chelator used to treat blood transfusion-related iron overload. Nephrotoxicity is the most serious and common adverse effect of deferasirox and may present as an acute or chronic kidney disease. However, scarce data are available on the molecular mechanisms of nephrotoxicity. We explored the therapeutic modulation of deferasirox-induced proximal tubular cell death in culture. Deferasirox induced dose-dependent tubular cell death and AnexxinV/7AAD staining showed features of apoptosis and necrosis...
January 31, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28101313/a-young-adult-with-unintended-acute-intravenous-iron-intoxication-treated-with-oral-chelation-the-use-of-liver-ferriscan-for-diagnosing-and-monitoring-tissue-iron-load
#14
Mohamed Yassin, Ashraf T Soliman, Vincenzo De Sanctis, Abbas Moustafa, Sandra Abou Samaan, Abdulqadir Nashwan
Acute iron intoxication (FeI) in humans has not been adequately studied. The manifestation of FeI, defined as a serum iron concentration >300 μg/dL (55 μmol/L) within 12 hours of ingestion, include various symptoms appearing in progressive stages. Systemic toxicity is expected with an intake of 60 mg/kg. A 27-year-old female nurse presented with unintended acute intravenous iron intoxication (FeI) a week after self-injecting herself with 20 ampoules of IV iron (4,000 mg elemental iron, 60 mg/kg). She had stable vital signs and mild hepatic tenderness...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28008230/efficacy-and-safety-of-iron-chelation-therapy-with-deferoxamine-deferiprone-and-deferasirox-for-the-treatment-of-iron-loaded-patients-with-nontransfusion-dependent-thalassemia-syndromes
#15
COMMENT
Ali T Taher, John B Porter, Antonis Kattamis, Vip Viprakasit, M Domenica Cappellini
No abstract text is available yet for this article.
2016: Drug Design, Development and Therapy
https://www.readbyqxmd.com/read/27994488/an-unfortunate-case-of-acquired-hemochromatosis-a-case-report-review-of-the-clinical-presentation-diagnosis-management-and-prognosis
#16
Anam Tariq, Kevin Westra, Arben Santo
BACKGROUND: While blood transfusions are commonly used for prophylaxis and treatment for acute chest syndromes and strokes in sickle cell patients, accumulation of excess iron resulting in secondary hemochromatosis remains a rare disease. Chelation is the mainstay for preventing and treating iron overload to deter potential end-organ damages; it is rare when therapy fails. CASE REPORT: A 52-year-old African American woman with chronic anemia secondary to sickle cell anemia and history of multiple blood transfusions presented with elevated serum ferritin (8000 ng/mL) and bilirubin (16...
2016: International Medical Case Reports Journal
https://www.readbyqxmd.com/read/27928480/a-review-on-iron-chelators-in-treatment-of-iron-overload-syndromes
#17
REVIEW
Naser Mobarra, Mehrnoosh Shanaki, Hassan Ehteram, Hajar Nasiri, Mehdi Sahmani, Mohsen Saeidi, Mehdi Goudarzi, Hoda Pourkarim, Mehdi Azad
Iron chelation therapy is used to reduce iron overload development due to its deposition in various organs such as liver and heart after regular transfusion. In this review, different iron chelators implicated in treatment of iron overload in various clinical conditions have been evaluated using more up-to-date studies focusing on these therapeutic agents. Deferoxamine, Deferiprone and Deferasirox are the most important specific US FDA-approved iron chelators. Each of these chelators has their own advantages and disadvantages, various target diseases, levels of deposited iron and clinical symptoms of the afflicted patients which may affect their selection as the best modality...
October 1, 2016: International Journal of Hematology-oncology and Stem Cell Research
https://www.readbyqxmd.com/read/27893351/management-of-iron-overload-in-myelodysplastic-syndromes-combined-deferasirox-and-deferoxamine-in-a-patient-with-liver-disease
#18
Claudio Cerchione, Giuseppe Cerciello, Simona Avilia, Roberta Della Pepa, Novella Pugliese, Marco Picardi, Lucio Catalano, Fabrizio Pane
No abstract text is available yet for this article.
October 24, 2016: Blood Transfusion, Trasfusione del Sangue
https://www.readbyqxmd.com/read/27893170/safety-profiles-of-iron-chelators-in-young-patients-with-haemoglobinopathies
#19
REVIEW
Sebastian Botzenhardt, Niya Li, Esther W Chan, Chor Wing Sing, Ian C K Wong, Antje Neubert
BACKGROUND: This review describes the safety of deferoxamine (DFO), deferiprone (DFP), deferasirox (DFX) and combined therapy in young patients less than 25 yr of age with haemoglobinopathies. METHODS: Searches in electronic literature databases were performed. Studies reporting adverse events associated with iron chelation therapy were included. Study and reporting quality was assessed using AHRQ Risk of Bias Assessment Tool and McMaster Quality Assessment Scale of Harms...
March 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/27881236/deferasirox-pharmacokinetics-evaluation-in-a-woman-with-hereditary-haemochromatosis-and-heterozygous-%C3%AE-thalassaemia
#20
Sarah Allegra, Silvia De Francia, Filomena Longo, Davide Massano, Jessica Cusato, Arianna Arduino, Elisa Pirro, Antonio Piga, Antonio D'Avolio
We present the deferasirox pharmacokinetics evaluation of a female patient on iron chelation, for the interesting findings from her genetic background (hereditary haemochromatosis and heterozygous β-thalassaemia) and clinical history (ileostomy; iron overload from transfusions). Drug plasma concentrations were measured by an HPLC-UV validated method, before and after ileum resection. Area under deferasirox concentration curve over 24h (AUC) values were determined by the mixed log-linear rule, using Kinetica software...
December 2016: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
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