Author/Editor     Glaser, Marjana; Glaser, Andrej
Title     Eritrocitoza
Translated title     Erythrocytosis
Type     članek
Source     In: Križman I, editor. Zbornik predavanj 23. strokovni sestanek internistov; 2011 sep 23-24; Ljubljana. Ljubljana: Združenje internistov SZD,
Publication year     2011
Volume     str. 113-20
Language     slo
Abstract     Erythrocytosis results when there is an increased red cell mass in circulation. It can be divided into primary intrinsic defects of the erythroid progenitor cell and secondary, for which factors external to the erythroid compartments are responsible. Erythrocytosis can be further divided into congenital (erythropoietin receptor mutations, defects of the oxygen sensing pathway) and acquired. There remains a small number of patients in whom no cause can be detected and the disease is defined as idiopahic erythrocytosis. Investigation for erythrocytosis should start with a full history and examination and to detect polycythemia vera (PRV) it is sufficient to determine beside elevated hematocrit the presence of JAK2 oncogen. In patients, who do not met the criteria for the diagnosis of PRV stage 1 and 2 investigations have to be preformed. Beside causes of erythrocytosis the clinical features and complications are presented and also guidelines of PRV treatment.
Summary     Eritrocitoza pomeni povečano maso eritrocitov v krvnem obtoku in je lahko posledica primarnih napak v eritroidni prednici ali pa so za njen nastanek odgovorni dejavniki izven eritroidnega oddelka. Eritrocitoza je prirojena (mutacije eritropetinskih receptorjev, motnje poti zaznavanja kisika) ali pridobljena. Pri majhni skupini bolnikov nikoli ne ugotovimo vzroka in bolezen opredelimo kot idiopatsko eritrocitozo. Potrebne preiskave za dokaz eritrocitoze vključujejo poleg anamneze in pregleda bolnika predvsem preiskave za diagnozo prave policitemije (PRV), kjer zadostuje poleg visokega hematokrita še dokaz JAK2 onkogena. Pri bolnikih, ki ne izpolnjujejo kriterijev za diagnozo PRV moramo opraviti stopenjsko diagnostiko. Poleg vzrokov eritrocitoze so predstavljeni klinični znaki in zapleti in predvsem smernice zdravljenja PRV.
Descriptors     POLYCYTHEMIA