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JFSF Vol 3, No 1, March 2018, p.13-25

doi: 10.22540/JFSF-03-013

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Review Article

A contemporary therapeutic approach to bone disease in beta-thalassemia - a review

Dimitrios Stefanopoulos1, Nikolaos A. Papaioannou1, Athanassios G. Papavassiliou2, George Mastorakos3, Andromachi Vryonidou4, Aikaterini Michou5, Ismene A. Dontas1, George Lyritis6, Eva Kassi2,7*, Symeon Tournis1*

  1. Laboratory for Research of the Musculoskeletal System “Th. Garofalidis”, KAT Hospital, National and Kapodistrian University of Athens, School of Medicine, Athens, Greece
  2. Department of Biological Chemistry, Medical School, National and Kapodistrian University of Athens, Athens, Greece
  3. Second Department of Obstetrics and Gynecology, National and Kapodistrian University of Athens, Aretaieion Hospital, Athens, Greece
  4. Department of Endocrinology, Diabetes & Metabolism, General Hospital Korgialenio-Benakio, Athens, Greece
  5. Deparment of Endocrinology, “Elena Venizelou” General Hospital, Athens, Greece
  6. Hellenic Osteoporosis Foundation, Athens, Greece
  7. First Department of Internal Medicine - Medical School- Laikon Hospital- National and Kapodistrian University of Athens, Greece
  • * Equally contribution

Keywords: Thalassemia, Osteoporosis, Bisphosphonates, Denosumab, Activin-A


Abstract

Homozygous beta-thalassemia represents a serious hemoglobinopathy, in which an amazing prolongation in the survival rate of patients has been achieved over recent decades. A result of this otherwise positive evolution is the fact that bone problems have become a major issue in this group of patients. Through an in-depth review of the related literature, the purpose of this study is to present and comment on the totality of the data that have been published to date pertaining to the prevention and treatment of thalassemia bone-disease, focusing on: the contribution of diet and lifestyle, the treatment of hematologic disease and its complications, the management of hypercalciuria, the role of vitamins and minerals and the implementation of anti-osteoporosis medical regimen. In order to comprehensively gather the above information, we mainly reviewed the international literature through the PubMed database, searching for the preventive and therapeutic data that have been published pertaining to thalassemia bone-disease over the last twenty-nine years. There is no doubt that thalassemia bone-disease is a complication of a multi-factorial etiopathology, which does not follow the rules of classical postmenopausal osteoporosis. Bisphosphonates have been the first line of treatment for many years now, with varied and usually satisfactory results. In addition, over the last few years, more data have arisen for the use of denosumab, teriparatide, and other molecules that are in the clinical trial phase, in beta-thalassemia.