Author/Editor     Domanovič, Dragoslav
Title     Hemokromatoza v tansfuzijski praksi
Translated title     Hemochromatosis in transfusion practice
Type     članek
Source     Med Razgl
Vol. and No.     Letnik 39, št. Suppl 5
Publication year     2000
Volume     str. 75-80
Language     slo
Abstract     Hemochromatosis is involved in transfusion practice in several ways. The basic treatment for primary hemochromatosis are therapeutic phlebotomies that are routinely performed at the transfusion centers. From 1997 at the Blood Transfusion Center in Ljubljana we performed 315 therapeutic phlebotomies for 18 male patients, that is on average 17.5 phlebotomies per patient. In 19 cases patients were deferred. In future we are expecting a higher number of patients and higher number of therapeutic phlebotomies. In some countries patients with primary hemochromatosis are accepted as regular blood donors and their blood can be used for transfusion to other patients. The decision was supported with the evidence that phlebotomy is safe for individuals with hemochromatosis and their blood could safely be used for transfusion. It is important to eliminate concern over donor motivation and modify regu- latory restrictions. With molecular genetic tests the bigger transfusion centers can coopera- te in diagnostics and research of hemochromatosis. Special components of young erythrocytes are in use for prevention of iron overload in patients with secondary hemochromatosis after transfusion.
Summary     Primarna hemokromatoza je povezana s transfuzijsko prakso na več načinov. Osnovno zdravljenje za primarno hemokromatozo so terapevtski odvzemi krvi, ki se pogosto izvajajo v transfuzijskih ustanovah. Od leta 1997 smo na Zavodu RS za transfuzijo krvi v Ljubljani pri 18 moških bolniških opravili 315 terapevtskih odvzemov krvi ali povprečno 17,5 odvzemov na bolnika. Bolnike smo odklonili v 19 primerih. V prihodnosti pričakujemo povečanje tako števila bolnikov kot rudi števila terapevtskih odvzemov krvi. Boolniki s hemokromatozo so v nekaterih državah uvrščeni med krvodajalce in je uporaba njihove krvi za transfuzijo drugim bolnikom dovoljena.. Odločitev so utemeljili z dokazi, da je odvzem krvi, ki poteka po merilih za krvodajalce, za bolnika s hemokromatozo popolnoma varen, njihova kri pa niškodljiva za zdravje prejemnikov transfuzije. Treba je zagovotiti pogoje, v katerih je lahko altruizem edini motivacijski dejavnik za dajanje krvi bolnikov s hemokromatozo, ter spremeniti in prilagoditi obstoječe normative, Večji transfuzijski centri rutinsko izvajajo sodobne molekularnogenetske teste, ki jim omogočajo sodelovanje v temeljnih in kliničnih ter populacijsko genetskih raziskavah primarne hemokromatoze. V transfuzijskih centrih se pripravljajo posebni pripravki eritrocitov za preprečevanje ali zmanjšanje nastanka sekundarne hemokromatoze pri kroničnih prejemnikih velikega števila transfuzij.
Descriptors     BLOOD TRANSFUSION
HEMOCHROMATOSIS
PHLEBOTOMY
BLOOD DONORS
HEMOSIDEROSIS