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Thalassemia major between liver and heart: Where we are now

Carlo Dessì1, GiovanBattista Leoni1, Paolo Moi2

  • 1Ospedale Regionale per le Microcitemie, ASL 8, Via Jenner, sn 09121 Cagliari, Italy.

Blood Cells, Molecules & Diseases
|May 16, 2015
PubMed

Insights

Thalassemia patients often experience iron overload in the heart and liver. Early combination therapy can reduce mortality, but ongoing monitoring for iron overload complications like cardiac disease and liver tumors remains crucial.

Area of Science:

  • Hematology
  • Cardiology
  • Hepatology

Background:

  • Thalassemia is a group of inherited blood disorders characterized by reduced hemoglobin production.
  • Iron overload is a common complication of regular blood transfusions required for thalassemia management.
  • Iron overload can lead to severe damage to vital organs, including the heart and liver.

Purpose of the Study:

  • To evaluate iron overload in the heart and liver among thalassemia patients.
  • To assess liver and heart-related morbidity and mortality in this cohort.
  • To identify factors influencing patient outcomes and complications.

Main Methods:

  • Retrospective analysis of a large cohort of thalassemia patients.
  • Assessment of myocardial and liver iron loading using clinical data.
  • Evaluation of cardiac deaths, morbidity, and liver stiffness (elastometry).
  • Analysis of treatment strategies, including combination therapy for heart failure.

Main Results:

  • Myocardial iron loading affected 28.9% of patients; 3.2% had severe loading.
  • Severe liver iron overload was observed in 15% of patients.
  • Cardiac deaths decreased significantly after 2003; combination therapy reduced short-term mortality.
  • Liver stiffness suggestive of cirrhosis was found in 16 HCVRNA positive and negative patients.
  • Rare liver tumors, including epithelioid hemangioendothelioma, were reported, necessitating transplantation.

Conclusions:

  • A subset of thalassemia patients develops progressive hemosiderosis, leading to cardiac disease and mortality.
  • Effective iron chelation is vital for preventing myocardial iron overload.
  • Liver iron chelation is essential for preventing hepatic tumors, not limited to hepatocarcinoma.

Keywords:
Combination therapyHemangioendotheliomaIron free fociIron overloadThalassemia major

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