Combined Liver and Heart Transplantation for End-Stage Iron-Induced Organ Failure in an Adult with Homozygous Beta-Thalassemia

Abstract

Most patients with homozygous β-thalassemia require red-cell transfusions to survive beyond the first decade of life. Although this intervention clearly prolongs survival, 1 it also results in the accumulation of iron in tissue, which is itself fatal without iron-chelating therapy 2 . Before the introduction of deferoxamine, iron-induced cardiac dysfunction was a predictable outcome in thalassemia, 3 and it is still the leading cause of death, followed in incidence by hepatic disease 1 . Despite the successes with deferoxamine, 4 – 7 many patients still have serious complications, because of either advanced age at the start of therapy or erratic compliance 8 – 14 . To date, the. © 1994, Massachusetts Medical Society. All rights reserved.