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Long-Term Trial of Deferiprone in 51 Transfusion-Dependent Iron
Overloaded Patients
A. Victor Hoffbrand,
Faris AL-Refaie,
Bernard Davis,
Noppadol Siritanakatkul,
Beverly F.A. Jackson,
John Cochrane,
Emma Prescott, and
Beatrix Wonke
From the Department of Hematology, The Royal Free Hospital School of
Medicine, London, UK; and the Departments of Hematology and Surgery,
Whittington Hospital, London, UK.
Fifty-one transfusion-dependent iron-loaded adult patients (38 with
thalassemia major) were treated with the orally active iron chelator
deferiprone (1,2 dimethyl-3-hydroxypyrid-4-one, L1) at a dose of 75 mg/kg/d (range, 50 to 79). Twenty patients discontinued the drug and
five died after a mean of 18.7 months (range, 4 to 35). Of the 20, 5 had arthropathy, 5 had gastrointestinal symptoms, 4 had a rising serum
ferritin, 3 had agranulocytosis or neutropenia, 1 had tachycardia, 1 had renal failure, and 1 went abroad. Twenty-six patients continued
deferiprone for a mean of 39.4 months (range, 12 to 49). Among these
patients, there was no overall significant change in serum ferritin
(initial mean, 2,937 µg/L; range, 980 to 5,970; final mean, 2,323 µg/L; range, 825 to 5,970) or in urine iron excretion (initial mean,
31.2 mg/24 h; range, 16.3 to 58.2; final mean, 32.1 mg/24 h; range, 9.4 to 75.8), implying no overall change in iron stores. When the patients who had received deferiprone for longer than 3 years were considered separately, there was also no significant change in serum ferritin or
urinary iron excretion. The initial serum ferritin levels in the 26 patients who continued deferiprone treatment were significantly lower
than in those who discontinued the drug (P < .01). The
liver iron content in 17 patients who had received deferiprone for 24 to 48 months ranged from 5.9 to 41.2 mg/g dry weight, 50% having levels above 15.0 mg, a level associated with a high risk of cardiac disease due to iron overload. In this study the drug caused fewer side
effects and was more effective at maintaining iron status among
patients previously well chelated and with lower initial serum ferritin
levels.
Blood, Vol. 91 No. 1 (January 1), 1998:
pp. 295-300
© 1998 by The American Society of Hematology.
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