Author/Editor     Marušič, Tamara; Černelč, Peter
Title     Vsebnost železa v telesu po transfuzijah krvi in zdravljenju z deferoksaminom
Translated title     Body iron stores after red blood cell transfusions and treatment with desferrioxamine
Type     članek
Source     Med Razgl
Vol. and No.     Letnik 39, št. Suppl 5
Publication year     2000
Volume     str. 81-4
Language     slo
Abstract     Background. Long-term treatment with red blood cell transfusions causes transfusional haemochromatosis. This complication can be prevented by subcutaneous or intravenous chelating therapy with 20-60 mg/kg desferrioxamine. This treatment is indicated in patients with anticipated longer survivals who require regular red blood cell transfusions. This study was undertaken to determine the amount of body iron in a group of treated with desferrioxamine during blood transfusion, and a group of patients receiving no desferrioxamine. Patients and methodss Body iron stores were measured in a group of 14 patients needing regular transfusions. They received intravenous infusion of 1 g desferrioxamine in 100 ml saline during every red blood cell transfusion, the overall number of transfusions for this group being 20 to 150 (median 78). A group of 8 patients who required less frequent red blood cell transfusions received no desferrioxamine. These patiens had a total of 11 to 58 (median 31.5) red blood cell transfusions. Results. Ferritin levels in the desferrioxamine group ranged from 460 to 9345 micro g/l (median 5384 micro g/l) and their transferrin saturation index was 76-92 (median 81.5). The group receiving no desferrioxamine during transfusions had ferritin levels from 133 to 10475 micro g/l (median 820.5 micro g/l) and a transferrin saturation index of 40-96 (median 71). Conclusion. Treatment with desferrioxamine is indicated in all patients requiring regular red blood cell transfusions. Negative iron balance, however, cannot be achieved by admini- stering desferrioxamine during red blood cell transfusions only. More frequent administration of desferrioxamine by the subcutaneous route, or treatment with the oral chelating agent deferiprone seem to be necessary to reduce excessive iron stores.
Summary     Izhodišča. Po dolgotrajnem zdravljenju s transfuzijami eritrocitov nastane posttransfuzijska hemokromatoza. To lahko preprečimo z rednim subkutanim ali intravenskim dajanjem helatov železa, kot je deferoksamin v odmerku 20 do 60 mg/kg. Z azdravljenje z njim se odločimo pri bolnikih s pričakovano daljšim preživetjem, ki potrebujejo pogoste transfuzije eristrocitov. Preveriti smo želeli vsebnost železa v telesu pri bolnikih, ki so med transfuzijami krvi prejemali deferoksamin, in pri tistih, ki ga niso prejemali. Bolniki in načini zdravljenja. Pri 14 bolnikih z boleznimi krvotvornih organov, ki potrebujejo transfuzije eritrocitov, smo z laboraorijskimi preiskavami ocenili količino železa v telesu. Šest bolnikov, ki je potrebovalo pogoste transfuzije krvi, je med vsako transfuzijo eritrocitov prejelo 1 g deferoksamina intravensko v 100 ml fiziološke rasztopine, Skupno so prejeli od 20 do 150 (mediana 78) enot koncentriranih eritrocitov. Osmim bolnikom, ki niso pogosto potrebovali transfuzije eritrocitov, deferoksamina nismo dajali. Skupno so prejeli od 11 do 58 (mediana 31,5) enot koncentriranih eritrocitov. Rezultati. Pri bolnikih, ki so prejeli veliko transfuzij eritrocitov in sočasno deferoksamin, je bila koncentracija feritina od 460 do 9345 mikro g/l (mediana 5384 mikro g/l), indeks nasičenosti trasnferina pa od 76 do 92 (mediana 81,5). Pri bolnikih, ki ob trasnfuzijah eritrocitov niso prejeli deferoksamina, je bila koncentracija feritina od 133 do 10475 mikro g/l (mediana 820,5 mikro g/l), indeks nasičenosti transferina pa od 40 do 96 (mediana 71). Zaključki. Zdravljenje z deferolsaminom je indicirano pri vseh bolnikih, ki potrebujejo pogoste transfuzije eritrocitov. Dajanje deferoksamina le ob transfuzijah krvi ni dovolj za zmanjšanje prevelike količine železa v telesu. Priporočeno bi bilo pogostejše subkutano dajanje deferoksamina ali peroralnega helatorja železa deferiprona.
Descriptors     ERYTHROCYTE TRANSFUSION
DEFEROXAMINE
HEMOSIDEROSIS