Transfusion therapy (Sickle-cell disease)
Transfusion therapy for sickle-cell disease entails the use of blood tranfusions in the management of acute cases of sickle cell disease and as a prophylaxis to prevent complications by decreasing the number of red blood cells (RBC) that can sickle by adding normal red blood cells.[1] In children prophylactic chronic red blood cell (RBC) transfusion therapy has been shown to be efficacious to a certain extent in reducing the risk of first stroke or silent stroke when transcranial Doppler (TCD) ultrasonography shows abnormal increased cerebral blood flow velocities. In those who have sustained a prior stoke event it also reduces the risk of recurrent stroke and additional silent strokes.[2][3]
Iron overload
Each unit of transfused blood has approximately 250 mg of iron, with each succesive transfusion, patients receiving chronic transfusion therapy accumulate iron in various tissues in the body as the body has no way to excrete the excess, this is a cause of increased morbidity and mortality.[4]The effects of iron overload are countered by chelation therapy[5]
References
- ^ Drasar E, Igbineweka N, Vasavda N, et al. Blood transfusion usage among adults with sickle cell disease - a single institution experience over ten years. Br J Haematol. 2011 Mar;152(6):766-70. doi: 10.1111/j.1365-2141.2010.08451.x. Epub 2011 Jan 31. PMID 21275951
- ^ Gyang E, Yeom K, Hoppe C, et al. Effect of chronic red cell transfusion therapy on vasculopathies and silent infarcts in patients with sickle cell disease'Am J Hematol. 2011 Jan;86(1):104-6. PMID 21117059
- ^ Mirre E, Brousse V, Berteloot L, et al. Feasibility and efficacy of chronic transfusion for stroke prevention in children with sickle cell disease Eur J Haematol. 2010 Mar;84(3):259-65. Epub 2009 Nov 12. PMID 19912310
- ^ Harmatz P, Butensky E, Quirolo K, et al. Severity of iron overload in patients with sickle cell disease receiving chronic red blood cell transfusion therapy. Blood. 2000 Jul 1;96(1):76-9. PMID 10891433
- ^ P. B. Walter, P. Harmatz, and E. Vichinsky, “Iron metabolism and iron chelation in sickle cell disease,” Acta Haematologica, vol. 122, no. 2-3, pp. 174–183, 2009.