Comparison of organ dysfunction in transfused patients with SCD or β thalassemia

Elliott Vichinsky, Ellen Butensky, Ellen Fung, Mark Hudes, Elizabeth Theil, Linda Ferrell, Roger Williams, Leslie Louie, Phillip D.K. Lee, Paul Harmatz

Research output: Contribution to journalReview article

104 Scopus citations

Abstract

Although it is life saving, transfusion therapy has resulted in the majority of sickle cell anemia and thalassemia patients being at risk for hemosiderosis-induced organ damage. It is unknown whether the complications of iron overload are affected by the underlying disease. In order to address this problem, we compared the prevalence of organ dysfunction in both groups of patients receiving chronic transfusion therapy (β thalassemia, N = 30; sickle cell anemia, N = 43). Both groups had similar quantitative liver iron. Thalassemia patients had greater cardiac disease (20% vs. 0%), growth failure (27% vs. 9%), and endocrine failure (37% vs. 0%). The strongest predictors of combined endocrine and cardiac disease in multivariate analysis were duration of chronic transfusion (P = 0.03) and diagnosis (P = 0.03). Quantitative liver iron concentration on a single liver biopsy was not predictive of cardiac or endocrine injury. Viral hepatitis is the strongest predictor of hepatocellular damage (P = 0.009), while the development of liver fibrosis is more closely related to liver iron concentration (P = 0.04). In conclusion, sickle cell anemia and thalassemia differ in the prevalence of organ injury. This difference is related to the duration of iron exposure and the specific hemoglobinopathy. A prospective study with a larger number of subjects is needed to confirm the relationships between specific diagnosis, liver iron concentration over time, and organ dysfunction.

Original languageEnglish (US)
Pages (from-to)70-74
Number of pages5
JournalAmerican Journal of Hematology
Volume80
Issue number1
DOIs
StatePublished - Sep 1 2005
Externally publishedYes

Keywords

  • Iron overload
  • Sickle cell anemia
  • Thalassemia
  • Transfusions

ASJC Scopus subject areas

  • Hematology

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    Vichinsky, E., Butensky, E., Fung, E., Hudes, M., Theil, E., Ferrell, L., Williams, R., Louie, L., Lee, P. D. K., & Harmatz, P. (2005). Comparison of organ dysfunction in transfused patients with SCD or β thalassemia. American Journal of Hematology, 80(1), 70-74. https://doi.org/10.1002/ajh.20402