Complications of β-thalassemia major in north America

被引:334
作者
Cunningham, MJ
Macklin, EA
Neufeld, EJ
Cohen, AR
机构
[1] Childrens Hosp, Dana Farber Canc Inst, Div Hematol, Boston, MA 02115 USA
[2] Harvard Univ, Sch Med, Boston, MA USA
[3] New England Res Inst, Watertown, MA 02172 USA
[4] Childrens Hosp Philadelphia, Philadelphia, PA 19104 USA
关键词
D O I
10.1182/blood-2003-09-3167
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Treatment of patients with beta-thalassemia major has improved dramatically during the past 40 years; however, the current clinical status of these patients remains poorly characterized. We performed a cross-sectional study of 342 patients in the Registry of the National Institutes of Health-sponsored Thalassemia Clinical Research Network. Evidence of hepatitis C exposure was present in 35% of tested patients, was associated with age, and had a rate of spontaneous viral clearance of 33%. Ferritin levels ranged from 147 to 11010 ng/mL (median, 1696 ng/mL). Median hepatic iron content was 7.8 mg/g dry weight and 23% of patients had values of 15 mg/g dry weight or higher. No patients 15 years or younger and 5% of patients aged 16 to 24 years had heart disease requiring medication. Ten percent had cirrhosis on biopsy. Endocrinologic complications were common among adults. Seventy-four (22%) patients had recent implantable central venous access devices (CVADs) placed. Among 80 episodes of bacteremia in 38 patients, 90% were attributable to the CVAD. Among 330 patients who had received deferoxamine chelation therapy, 224 (68%) reported no complications. We conclude that hepatitis C, iron-related organ dysfunction, and complications of iron chelation therapy are strongly age-dependent in North American patients with beta-thalassemia. (C) 2004 by The American Society of Hematology.
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页码:34 / 39
页数:6
相关论文
共 32 条
[1]  
[Anonymous], 2002, MMWR MORB MORTAL WKL, V51, P973
[2]   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
[3]   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
[4]  
*CDCP, 2002, JAMA-J AM MED ASSOC, V288, P2535
[5]   Vertical transmission of hepatitis C virus in a cohort of 2,447 HIV-seronegative pregnant women: A 24-month prospective study [J].
Ceci, O ;
Margiotta, T ;
Marello, F ;
Francavilla, R ;
Loizzi, P ;
Francavilla, A ;
Mautone, A ;
Impedovo, L ;
Ierardi, E ;
Mastroianni, M ;
Bettocchi, S ;
Selvaggi, L .
JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION, 2001, 33 (05) :570-575
[6]   Association between HLA class II genotype and spontaneous clearance of hepatitis C viraemia [J].
Cramp, ME ;
Carucci, P ;
Underhill, J ;
Naoumov, NV ;
Williams, R ;
Donaldson, PT .
JOURNAL OF HEPATOLOGY, 1998, 29 (02) :207-213
[7]   Long-term outcome of continuous 24-hour deferoxamine infusion via indwelling intravenous catheters in high-risk β-thalassemia [J].
Davis, BA ;
Porter, JB .
BLOOD, 2000, 95 (04) :1229-1236
[8]   Growth and puberty and its management in thalassaemia [J].
De Sanctis, V .
HORMONE RESEARCH, 2002, 58 :72-79
[9]   Results of long-perm iron-chelating therapy [J].
Gabutti, V ;
Piga, A .
ACTA HAEMATOLOGICA, 1996, 95 (01) :26-36
[10]   Iron chelators for thalassaemia [J].
Hershko, C ;
Konijn, AM ;
Link, G .
BRITISH JOURNAL OF HAEMATOLOGY, 1998, 101 (03) :399-406