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Blood, Vol. 95 No. 4 (February 15), 2000:
pp. 1229-1236
Long-term outcome of continuous 24-hour deferoxamine infusion
via indwelling intravenous catheters in high-risk  -thalassemia
Bernard A. Davis and
John B. Porter
From the Department of Hematology, University College London Medical
School, London, England.
The optimal regimen of intravenous deferoxamine for iron overload in
high-risk homozygous  -thalassemia is unknown because only short-term
follow-up has been described in small patient groups. We report the
outcome over a 16-year period of a continuous 24-hour deferoxamine
regimen, with dose adjustment for serum ferritin, delivered via 25 indwelling intravenous lines for 17 patients. Treatment indications
were cardiac arrhythmias, left ventricular dysfunction, gross iron
overload, and intolerability of subcutaneous deferoxamine. Cardiac
arrhythmias were reversed in 6 of 6 patients, and the left ventricular
ejection fraction improved in 7 of 9 patients from a mean (± SEM) of
36 ± 2% to 49 ± 3% (P = .002, n = 9). The
serum ferritin fell in a biphasic manner from a pretherapy mean of
6281 ± 562 µg/L to 3736 ± 466 µg/L (P = .001),
falling rapidly and proportionally to the pretreatment ferritin
(r2 = 0.99) for values >3000 µg/L
but falling less rapidly below this value (at 133 ± 22 µg/L/mo).
The principal catheter-related complications were
infection and thromboembolism (1.15 and 0.48 per 1000 catheter days,
respectively), rates similar to other patient groups. Only one case of
reversible deferoxamine toxicity was observed (retinal) when the
therapeutic index was briefly exceeded. An actuarial survival of 61%
at 13 years with no treatment-related mortality provides evidence of
the value of this protocol.
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