Purging iron from the heart

被引:32
作者
Hershko, C
Cappellini, MD
Galanello, R
Piga, A
Tognoni, G
Masera, G
机构
[1] Hebrew Univ Jerusalem, Hadassah Med Sch, IL-91010 Jerusalem, Israel
[2] Ben Gurion Univ Negev, Fac Med, IL-84105 Beer Sheva, Israel
[3] Osped Policlin Milano, Ctr Congenital Anemia, Milan, Italy
[4] Dept Biomed Sci & Biotechnol, Cagliari, Italy
[5] Univ Turin, Dept Pediat Hematol, I-10124 Turin, Italy
[6] Ist Ric Farmacol Mario Negri, Consorzio Mario Negri Sud, I-66030 Santa Maria Imbaro, Italy
[7] Univ Milano Bicocca, Osp San Gerardo, Pediat Clin, Monza, Italy
关键词
iron chelation; haemosiderosis; heart; desferrioxamine; deferiprone;
D O I
10.1111/j.1365-2141.2004.04946.x
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Methods are now available to measure the magnitude of iron accumulation in the heart. Their validation currently relies on indirect evidence and not on chemical estimation in cardiac biopsies. All patients with symptomatic heart disease appear to have abnormal T2* values, but many patients without symptomatic heart disease also have evidence of increased myocardial iron. Although there is no proof to date that increased myocardial iron, as evidenced by abnormal magnetic resonance imaging, carries an adverse prognosis, it is likely that such new information will affect the chelating programme of patients. In these cases, there are a number of options available: (i) ongoing treatment with either desferrioxamine (DFO) or deferiprone may be intensified; (ii) the patient may be switched to the alternative chelator or (iii) combined chelation with both DFO and deferiprone may be started, which is more effective than using either chelator alone. For patients with symptomatic heart disease, continuous intravenous DFO with, or without deferiprone, remains the currently recommended treatment, in view of its documented ability to salvage these patients.
引用
收藏
页码:545 / 551
页数:7
相关论文
共 48 条
  • [1] ALDOURI MA, 1990, ACTA HAEMATOL-BASEL, V84, P113
  • [2] PHARMACOKINETICS OF THE ORAL IRON CHELATOR DEFERIPRONE (L(1)) IN PATIENTS WITH IRON OVERLOAD
    ALREFAIE, FN
    SHEPPARD, LN
    NORTEY, P
    WONKE, B
    HOFFBRAND, AV
    [J]. BRITISH JOURNAL OF HAEMATOLOGY, 1995, 89 (02) : 403 - 408
  • [3] Comparison of effects of oral deferiprone and subcutaneous desferrioxamine on myocardial iron concentrations and ventricular function in beta-thalassaemia
    Anderson, LJ
    Wonke, B
    Prescott, E
    Holden, S
    Walker, JM
    Pennell, DJ
    [J]. LANCET, 2002, 360 (9332) : 516 - 520
  • [4] Cardiovascular T2-star (T2*) magnetic resonance for the early diagnosis of myocardial iron overload
    Anderson, LJ
    Holden, S
    Davis, B
    Prescott, E
    Charrier, CC
    Bunce, NH
    Firmin, DN
    Wonke, B
    Porter, J
    Walker, JM
    Pennell, DJ
    [J]. EUROPEAN HEART JOURNAL, 2001, 22 (23) : 2171 - 2179
  • [5] Survival and disease complications in thalassemia major
    Borgna-Pignatti, C
    Rugolotto, S
    De Stefano, P
    Piga, A
    Di Gregorio, F
    Gamberini, MR
    Sabato, V
    Melevendi, C
    Cappellini, MD
    Verlato, G
    [J]. COOLEYS ANEMIA: SEVENTH SYMPOSIUM, 1998, 850 : 227 - 231
  • [6] MAGNETIC-SUSCEPTIBILITY MEASUREMENT OF HUMAN IRON STORES
    BRITTENHAM, GM
    FARRELL, DE
    HARRIS, JW
    FELDMAN, ES
    DANISH, EH
    MUIR, WA
    TRIPP, JH
    BELLON, EM
    [J]. NEW ENGLAND JOURNAL OF MEDICINE, 1982, 307 (27) : 1671 - 1675
  • [7] EFFICACY OF DEFEROXAMINE IN PREVENTING COMPLICATIONS OF IRON OVERLOAD IN PATIENTS WITH THALASSEMIA MAJOR
    BRITTENHAM, GM
    GRIFFITH, PM
    NIENHUIS, AW
    MCLAREN, CE
    YOUNG, NS
    TUCKER, EE
    ALLEN, CJ
    FARRELL, DE
    HARRIS, JW
    [J]. NEW ENGLAND JOURNAL OF MEDICINE, 1994, 331 (09) : 567 - 573
  • [8] IRON IN HEART - ETIOLOGY AND CLINICAL SIGNIFICANCE
    BUJA, LM
    ROBERTS, WC
    [J]. AMERICAN JOURNAL OF MEDICINE, 1971, 51 (02) : 209 - &
  • [9] Survival and morbidity in transfusion-dependent thalassemic patients on subcutaneous desferrioxamine chelation - Nearly two decades of experience
    Calleja, EM
    Shen, JY
    Lesser, M
    Grady, RW
    New, MI
    Giardina, PJ
    [J]. COOLEYS ANEMIA: SEVENTH SYMPOSIUM, 1998, 850 : 469 - 470
  • [10] The safety and effectiveness of deferiprone in a large-scale, 3-year study in Italian patients
    Ceci, A
    Baiardi, P
    Felisi, M
    Cappellini, MD
    Carnelli, V
    De Sanctis, V
    Galanello, R
    Maggio, A
    Masera, G
    Piga, A
    Schettini, F
    Stefàno, I
    Tricta, F
    [J]. BRITISH JOURNAL OF HAEMATOLOGY, 2002, 118 (01) : 330 - 336