Survival and disease complications in thalassemia major

Ann N Y Acad Sci. 1998 Jun 30:850:227-31. doi: 10.1111/j.1749-6632.1998.tb10479.x.

Abstract

We studied survival and disease complications in 1,146 patients with thalassemia major, born from January 1, 1960 to December 31, 1987. At last follow-up, in March 1997, probability of survival to age 20 years was 89% and to age 25 years was 82% for patients born in the years 1970-1974. Patients who died had a serum ferritin level, measured the year before death, significantly higher than those who survived. Diabetes was present in 5.4% of the patients; heart failure in 6.4%; arrhythmias in 5.0%, thrombosis in 1.1%, hypothyroidism in 11.6%, HIV infection in 1.8%. Hypogonadism was diagnosed in 55% of 578 patients who had reached pubertal age: 83.5% of hypogonadic females and 78.6% of males were receiving substitutive hormonal therapy. In conclusion, the survival of patients with thalassemia major is good and improving, but the prevalence of severe complications is still high.

Publication types

  • Multicenter Study

MeSH terms

  • Adolescent
  • Adult
  • Cause of Death
  • Child
  • Cohort Studies
  • Diabetes Mellitus / epidemiology
  • Disease-Free Survival
  • Female
  • Follow-Up Studies
  • Heart Diseases / epidemiology
  • Heart Diseases / mortality
  • Humans
  • Hypogonadism / epidemiology
  • Italy
  • Male
  • Probability
  • Sex Factors
  • Survival Rate
  • Time Factors
  • beta-Thalassemia / complications*
  • beta-Thalassemia / mortality
  • beta-Thalassemia / therapy*