Malik_tab3: Mainstay of symptomatic therapy of transfusion-dependent thalassaemia

Expert Reviews in Molecular Medicine: http://www.expertreviews.org/
Accession information: Vol. 8; Issue 10; 9 May 2006 Abstract
PDF

Mainstay of symptomatic therapy of transfusion-dependent thalassaemia

Fabrizia Urbinati, Catherine Madigan and Punam Malik

Author contact details

Table 3. Mainstay of symptomatic therapy of transfusion-dependent thalassaemia
Treatment	Goal
RBC transfusions every 2–3 weeks to maintain a pre-transfusion Hb concentration of 9–10 g/dL	Treatment of anaemia and tissue hypoxia: improvement of growth and development; suppression of medullary/extramedullary haematopoiesis and therefore hepatosplenomegaly, hypersplenism and bone deformities
Serum ferritin, liver biopsy, Cardiac T2* MRI (Ref. 227) Daily subcutaneous DFO infusions Decreased iron and increased tea intake	Iron overload management: regular monitoring for iron status; chelation of iron and increased excretion; decreased gut absorption of iron
Hormonal levels and function (pituitary, gonadal, pancreatic) Adequate transfusion and chelation Hormonal replacement Assisted reproductive techniques	Monitoring and maintenance of normal endocrine function and fertility
Increased calcium and vitamin D supplementation Bisphosphonates; careful monitoring of chelation	Bone-disease management: ensuring adequate mineralisation of bones, and reducing bone resorption (Refs 4, 228, 229)
Abbreviations: DFO, desferrioxamine; Hb, haemoglobin; MRI, magnetic resonance imaging; RBC, red blood cell.

References cited in Table 1

4 Rund, D. and Rachmilewitz, E. (2005) Beta-thalassemia. N Engl J Med 353, 1135-1146, PubMed

227 Anderson, L.J. et al. (2001) Cardiovascular T2-star (T2*) magnetic resonance for the early diagnosis of myocardial iron overload. Eur Heart J 22, 2171-2179, PubMed

228 Wonke, B. et al. (1998) Genetic and acquired predisposing factors and treatment of osteoporosis in thalassaemia major. J Pediatr Endocrinol Metab 11 Suppl 3, 795-801, PubMed

229 Wonke, B. (1998) Bone disease in beta-thalassaemia major. Br J Haematol 103, 897-901, PubMed

| home | search | glossary | links | sitemap | contact |

Expert Reviews in Molecular Medicine © Cambridge University Press ISSN 1462-3994 (Disclaimer and copyright)
Editorial Office: Centre for Applied Research in Educational Technologies (CARET), 1st Floor, 16 Mill Lane, Cambridge, CB2 1SB, UK. Tel: +44 (0)1223 765 375; Fax: +44(0)1223 765 505; E-mail: ermm@caret.cam.ac.uk