Effective combination therapy of deferiprone and deferoxamine for the rapid clearance of excess cardiac iron and the prevention of heart disease in thalassemia. The protocol of the international committee on oral chelators.
International Committee on Oral Chelators (ICOC) protocol;
chelation therapy;
deferiprone (L1);
deferoxamine (DFO);
combination therapy;
thalassemia;
iron overload;
D O I:
10.1080/03630260600642567
中图分类号:
Q5 [生物化学];
Q7 [分子生物学];
学科分类号:
071010 ;
081704 ;
摘要:
The International Committee on Oral Chelators (ICOC) combination therapy protocol involving the administration of deferiprone (L1) during the day (80-110 mg/kg/day) and deferoxamine (DFO) (40-60 mg/kg at least 3 days/week) during the night for 8-12 hours using a pump, or the whole 24 hours using an elastomeric pump infuser, has been tested in 11 thalassemia patients (seven males, four females) over a period of 9-28nionths. The patients had variable serum ferritin levels (0.54-4.6 mg/L) and cardiac iron load ranging from normal to severe siderosis levels (MRI T2*: 4.7-45 ms). There was a substantial overall reduction in serum ferritin levels (0.17-2.16 mg/L) and. normalization of cardiac iron (MRI T2* > 20 ms) in all patients. In two patients with severe and moderate cardiac iron load range levels, cardiac iron normalization was achieved within 9-10 months. Two patients on L1 monotherapy (80-120 mg/kg/day) maintained normal range MRI T2* cardiac iron levels over the same period. The ICOC combination therapy protocol appears to be the most effective and least cumbersome form of chelation treatment for the rapid clearance of excess iron from the heart.
机构:Royal Brompton Hosp, Cardiovasc Magnet Resonance Unit, London SW3 6NP, England
Anderson, LJ
;
Wonke, B
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机构:Royal Brompton Hosp, Cardiovasc Magnet Resonance Unit, London SW3 6NP, England
Wonke, B
;
Prescott, E
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机构:Royal Brompton Hosp, Cardiovasc Magnet Resonance Unit, London SW3 6NP, England
Prescott, E
;
Holden, S
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机构:Royal Brompton Hosp, Cardiovasc Magnet Resonance Unit, London SW3 6NP, England
Holden, S
;
Walker, JM
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机构:Royal Brompton Hosp, Cardiovasc Magnet Resonance Unit, London SW3 6NP, England
Walker, JM
;
Pennell, DJ
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机构:
Royal Brompton Hosp, Cardiovasc Magnet Resonance Unit, London SW3 6NP, EnglandRoyal Brompton Hosp, Cardiovasc Magnet Resonance Unit, London SW3 6NP, England
机构:
Cornell Univ, New York Presbyterian Hosp, Weill Med Coll, Div Pediat Hematol Oncol, New York, NY 10021 USACornell Univ, New York Presbyterian Hosp, Weill Med Coll, Div Pediat Hematol Oncol, New York, NY 10021 USA
Giardina, PJ
;
Grady, RW
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机构:
Cornell Univ, New York Presbyterian Hosp, Weill Med Coll, Div Pediat Hematol Oncol, New York, NY 10021 USACornell Univ, New York Presbyterian Hosp, Weill Med Coll, Div Pediat Hematol Oncol, New York, NY 10021 USA
机构:Royal Brompton Hosp, Cardiovasc Magnet Resonance Unit, London SW3 6NP, England
Anderson, LJ
;
Wonke, B
论文数: 0引用数: 0
h-index: 0
机构:Royal Brompton Hosp, Cardiovasc Magnet Resonance Unit, London SW3 6NP, England
Wonke, B
;
Prescott, E
论文数: 0引用数: 0
h-index: 0
机构:Royal Brompton Hosp, Cardiovasc Magnet Resonance Unit, London SW3 6NP, England
Prescott, E
;
Holden, S
论文数: 0引用数: 0
h-index: 0
机构:Royal Brompton Hosp, Cardiovasc Magnet Resonance Unit, London SW3 6NP, England
Holden, S
;
Walker, JM
论文数: 0引用数: 0
h-index: 0
机构:Royal Brompton Hosp, Cardiovasc Magnet Resonance Unit, London SW3 6NP, England
Walker, JM
;
Pennell, DJ
论文数: 0引用数: 0
h-index: 0
机构:
Royal Brompton Hosp, Cardiovasc Magnet Resonance Unit, London SW3 6NP, EnglandRoyal Brompton Hosp, Cardiovasc Magnet Resonance Unit, London SW3 6NP, England
机构:
Cornell Univ, New York Presbyterian Hosp, Weill Med Coll, Div Pediat Hematol Oncol, New York, NY 10021 USACornell Univ, New York Presbyterian Hosp, Weill Med Coll, Div Pediat Hematol Oncol, New York, NY 10021 USA
Giardina, PJ
;
Grady, RW
论文数: 0引用数: 0
h-index: 0
机构:
Cornell Univ, New York Presbyterian Hosp, Weill Med Coll, Div Pediat Hematol Oncol, New York, NY 10021 USACornell Univ, New York Presbyterian Hosp, Weill Med Coll, Div Pediat Hematol Oncol, New York, NY 10021 USA