Cardiac morbidity and mortality in deferoxamine- or deferiprone-treated patients with thalassemia major

被引:300
作者
Borgna-Pignatti, C
Cappellini, MD
De Stefano, P
Del Vecchio, GC
Forni, GL
Gamberini, MR
Ghilardi, R
Piga, A
Romeo, MA
Zhao, HQ
Cnaan, A
机构
[1] Univ Ferrara, Pediat Clin, Dipartimento Med Clin & Sperimentale, I-44100 Ferrara, Italy
[2] Univ Turin, Pediat Clin, Turin, Italy
[3] Univ Catania, Pediat Clin, Catania, Italy
[4] Univ Milan, Pediat Clin, Milan, Italy
[5] Univ Bari, Pediat Clin, Bari, Italy
[6] IRCCS, Policlin San Matteo, Pavia, Italy
[7] Osped Galliera, Genoa, Italy
[8] IRCCS, Ctr Anemie Congenite, Osped Maggiore Policlin, Milan, Italy
[9] Univ Penn, Sch Med, Childrens Hosp Philadelphia, Div Biostat & Epidemiol, Philadelphia, PA 19104 USA
关键词
D O I
10.1182/blood-2005-07-2933
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Deferoxamine (DFO) therapy has been associated with improved survival of thalassemia patients. However, cardiac disease remains the main cause of death in those patients. In 1995, the oral chelator deferiprone became available for clinical use. We compared the occurrence of cardiac disease in patients treated only with DFO and in those whose therapy was switched to deferiprone during the period of observation, from January 31, 1995, to December 31, 2003. All patients with thalassemia major treated in 7 Italian centers who were born between 1970 and 1993 and who had not experienced a cardiac event prior to January 1995 were included. DFO only was given to 359 patients, and 157 patients received deferiprone for part of the time. A total of 3610 patient-years were observed on DFO and 750 on deferiprone. At baseline, the 2 groups were comparable for age and sex, while ferritin levels were significantly higher in patients switched to deferiprone. Fifty-two cardiac events, including 10 cardiac deaths, occurred during therapy with DFO. No cardiac events occurred during deferiprone therapy or within at least 18 months after the end of it. In the setting of a natural history study, deferiprone therapy was associated with significantly greater cardiac protection than deferoxamine in patients with thalassemia major.
引用
收藏
页码:3733 / 3737
页数:5
相关论文
共 16 条
[1]   PHARMACOKINETICS OF THE ORAL IRON CHELATOR DEFERIPRONE (L(1)) IN PATIENTS WITH IRON OVERLOAD [J].
ALREFAIE, FN ;
SHEPPARD, LN ;
NORTEY, P ;
WONKE, B ;
HOFFBRAND, AV .
BRITISH JOURNAL OF HAEMATOLOGY, 1995, 89 (02) :403-408
[2]   Comparison of effects of oral deferiprone and subcutaneous desferrioxamine on myocardial iron concentrations and ventricular function in beta-thalassaemia [J].
Anderson, LJ ;
Wonke, B ;
Prescott, E ;
Holden, S ;
Walker, JM ;
Pennell, DJ .
LANCET, 2002, 360 (9332) :516-520
[3]   Survival and disease complications in thalassemia major [J].
Borgna-Pignatti, C ;
Rugolotto, S ;
De Stefano, P ;
Piga, A ;
Di Gregorio, F ;
Gamberini, MR ;
Sabato, V ;
Melevendi, C ;
Cappellini, MD ;
Verlato, G .
COOLEYS ANEMIA: SEVENTH SYMPOSIUM, 1998, 850 :227-231
[4]  
Borgna-Pignatti C, 2004, HAEMATOLOGICA, V89, P1187
[5]  
Breslow NE, 1980, IARC SCI PUBLICATION, V32
[6]   The safety and effectiveness of deferiprone in a large-scale, 3-year study in Italian patients [J].
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 .
BRITISH JOURNAL OF HAEMATOLOGY, 2002, 118 (01) :330-336
[7]  
Cohen AR, 2000, BRIT J HAEMATOL, V108, P305
[8]  
COLLET D, 2003, MODELLING SURVIVAL D, P160
[9]   The iron-loaded gerbil model revisited: Effects of deferoxamine and deferiprone treatment [J].
Hershko, C ;
Link, G ;
Konijn, AM ;
Huerta, M ;
Rosenmann, E ;
Reinus, C .
JOURNAL OF LABORATORY AND CLINICAL MEDICINE, 2002, 139 (01) :50-58
[10]  
HERSHKO C, 1991, BLOOD, V77, P2049