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Previous Volume 331:567-573 September 1, 1994 Number 9 Next

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ABSTRACT

Background To determine whether deferoxamine prevents the complications of transfusional iron overload in thalassemia major, we evaluated 59 patients (30 were female and 29 male; age range, 7 to 31 years) periodically for 4 to 10 years or until death.

Methods At each follow-up visit, we performed a detailed clinical and laboratory evaluation and measured hepatic iron stores with a noninvasive magnetic device.

Results The body iron burden as assessed by magnetic measurement of hepatic iron stores was closely correlated (R = 0.89, P<0.001) with the ratio of cumulative transfusional iron load to cumulative deferoxamine use (expressed in millimoles of iron per kilogram of body weight, in relation to grams of deferoxamine per kilogram, transformed into the natural logarithm). Each increase of one unit in the natural logarithm of the ratio (transfusional iron load to deferoxamine use) was associated with an increased risk of impaired glucose tolerance (relative risk, 19.3; 95 percent confidence interval, 4.8 to 77.4), diabetes mellitus (relative risk, 9.2; 95 percent confidence interval, 1.8 to 47.7), cardiac disease (relative risk, 9.9; 95 percent confidence interval, 1.9 to 51.2), and death (relative risk, 12.6; 95 percent confidence interval, 2.4 to 65.4). All nine deaths during the study occurred among the 23 patients who had begun chelation therapy later and used less deferoxamine in relation to their transfusional iron load (P<0.001).

Conclusions The early use of deferoxamine in an amount proportional to the transfusional iron load reduces the body iron burden and helps protect against diabetes mellitus, cardiac disease, and early death in patients with thalassemia major.

Source Information

From the Departments of Medicine (G.M.B., J.W.H.) and Physics (C.J.A., D.E.F.), Case Western Reserve University, Cleveland; the Department of Mathematics, Moorhead State University, Moorhead, Minn. (C.E.M.); St. Jude Children's Research Hospital, Memphis, Tenn. (A.W.N.); and the Clinical Hematology Branch (P.M.G., N.S.Y.) and Cardiology Branch (E.E.T.), National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Md.

Address reprint requests to Dr. Brittenham at MetroHealth Medical Center, 3395 Scranton Rd., Cleveland, OH 44109.

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Deferoxamine in Thalassemia Major
Splendiani G., Tozzo C., Mazzarella V., Casciani C. U., Lucarelli G., Clift R., Angelucci E., Cazzola M., Locatelli F., De Stefano P., Olivieri N. F., Nathan D. G., Cohen A. R.
Extract | Full Text  
N Engl J Med 1995; 332:270-273, Jan 26, 1995. Correspondence

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