Effective Iron Chelation Practice for Patients With β-Thalassemia Major

被引:3
作者
Carson, Susan M. [1 ,2 ]
Martin, Marie B. [3 ]
机构
[1] Childrens Hosp Los Angeles, Thalassemia & Chron Transfus Program, Los Angeles, CA 90027 USA
[2] Univ Calif Los Angeles, Los Angeles, CA USA
[3] Childrens Hosp Philadelphia, Philadelphia, PA USA
关键词
chronic illnesses/comorbidities; fatigue; leukemia; lymphomas; hematology; MYOCARDIAL IRON; MAGNETIC-RESONANCE; CARDIAC IRON; LIVER IRON; OVERLOAD; DEFEROXAMINE; DEFERASIROX; CARDIOMYOPATHY; TRANSFUSION; MANAGEMENT;
D O I
10.1188/14.CJON.102-111
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
Chronic blood transfusion is the only treatment for severe anemia in patients with beta-thalassemia major. However,, red blood cell transfusions lead to iron overload and subsequent organ damage because of the toxic. effects of iron. The heart is particularly vulnerable to iron toxicity, and heart failure is the leading cause of death-among these patients Iron chelation therapy prevents or reverses iron loading, thereby reducing the risk of complications from excess iron. Serum ferritin and liver iron concentration often are used to gauge the risk of organ iron overload; but these Measurements may not correlate well with cardiac iron load. Magnetic resonance imaging (MRI) is a noninvasive diagnostic tool that can provide a more direct measure of iron concentration in both the heart and liven Cardiac iron determined by MRI is expressed as a function of T2*, in which higher values represent lower concentrations. Changes in T2* are used to assess the effectiveness of iron chelation and to adjust therapy. Early treatment and compliance are keys to successful therapy. Nursing strategies to optimize chelation therapy include identifying, patients who are at risk for developing, organ damage, developing,chelation, plans, promoting compliance, and educating patients. The efficacy and safety of iron chelators, as well as,nursing best practices, are reviewed.
引用
收藏
页码:102 / 111
页数:10
相关论文
共 47 条
[1]   Cardiac magnetic resonance imaging R2*assessments and analysis of historical parameters in patients with transfusion-dependent thalassernia [J].
Aessopos, Athanassios ;
Fragodimitri, Christina ;
Karabatsos, Fotios ;
Hatziliami, Antonia ;
Yousef, Jacqueline ;
Giakoumis, Anastasios ;
Dokou, Aikaterini ;
Gotsis, Efstathios D. ;
Berdoukas, Vasilis ;
Karagiorga, Markissia .
HAEMATOLOGICA-THE HEMATOLOGY JOURNAL, 2007, 92 (01) :131-132
[2]   Cardiovascular T2-star (T2*) magnetic resonance for the early diagnosis of myocardial iron overload [J].
Anderson, LJ ;
Holden, S ;
Davis, B ;
Prescott, E ;
Charrier, CC ;
Bunce, NH ;
Firmin, DN ;
Wonke, B ;
Porter, J ;
Walker, JM ;
Pennell, DJ .
EUROPEAN HEART JOURNAL, 2001, 22 (23) :2171-2179
[3]   Myocardial iron clearance during reversal of siderotic cardiomyopathy with intravenous desferrioxamine: a prospective study using T2*cardiovascular magnetic resonance [J].
Anderson, LJ ;
Westwood, MA ;
Holden, S ;
Davis, B ;
Prescott, E ;
Wonke, B ;
Porter, JB ;
Walker, JM ;
Pennell, DJ .
BRITISH JOURNAL OF HAEMATOLOGY, 2004, 127 (03) :348-355
[4]   Development of thalassaemic iron overload cardiomyopathy despite low liver iron levels and meticulous compliance to desferrioxamine [J].
Anderson, LJ ;
Westwood, MA ;
Prescott, E ;
Walker, JM ;
Pennell, DJ ;
Wonke, B .
ACTA HAEMATOLOGICA, 2006, 115 (1-2) :106-108
[5]   Medical progress: Disorders of iron metabolism [J].
Andrews, NC .
NEW ENGLAND JOURNAL OF MEDICINE, 1999, 341 (26) :1986-1995
[6]   Italian Society of Hematology practice guidelines for the management of iron overload in thalassemia major and related disorders [J].
Angelucci, Emanuele ;
Barosi, Giovanni ;
Camaschella, Clara ;
Cappellini, Maria Domenica ;
Cazzola, Mario ;
Galanello, Renzo ;
Marchetti, Monia ;
Piga, Antonio ;
Tura, Sante .
HAEMATOLOGICA, 2008, 93 (05) :741-752
[7]  
[Anonymous], 2011, FERR PACK INS
[8]   Understanding Iron Overload: Screening, Monitoring, and Caring for Patients With Transfusion-Dependent Anemias [J].
Ault, Patricia ;
Jones, Karen .
CLINICAL JOURNAL OF ONCOLOGY NURSING, 2009, 13 (05) :511-517
[9]  
Borgna-Pignatti C, 2004, HAEMATOLOGICA, V89, P1187
[10]   EFFICACY OF DEFEROXAMINE IN PREVENTING COMPLICATIONS OF IRON OVERLOAD IN PATIENTS WITH THALASSEMIA MAJOR [J].
BRITTENHAM, GM ;
GRIFFITH, PM ;
NIENHUIS, AW ;
MCLAREN, CE ;
YOUNG, NS ;
TUCKER, EE ;
ALLEN, CJ ;
FARRELL, DE ;
HARRIS, JW .
NEW ENGLAND JOURNAL OF MEDICINE, 1994, 331 (09) :567-573