IZOLIRANA APLAZIJA CRVENE KRVNE LOZE U SISTEMSKOM ERITEMSKOM LUPUSU – PRIKAZ BOLESNICE

Marija Tomac-Stojmenović, Ita Hadžisejdić, Toni Valković, Srđan Novak

Acquired pure red cell aplasia (PRCA) is a rare, generally chronic condition of profound anemia characterized by a severe reduction in the number of reticulocytes in the peripheral blood and the virtual absence  of erythroid precursors in the bone marrow. All other cell lineages are present and appear morphologically normal. In systemic lupus erythematosus (SLE) red cell aplasia is probably due to antibodies directed against either erythropoietin or bone marrow erythroblasts. Th is form of anemia usually responds to steroids, although cyclophosphamide and cyclosporine have been successfully employed as well. We present the case of a patient with PRCA in SLE.

Stečena aplazija crvene krvne loze (engl. Pure red cell aplasia – PRCA) rijedak je poremećaj najčešće kroničnog tijeka. Karakteriziran je teškom anemijom uz retikulocitopeniju u perifernoj krvi te odsutnost eritroblasta u koštanoj srži. Ostale linije krvotvornih stanica u koštanoj srži morfološki su uredne. U sistemskom eritemskom lupusu pojava PRCA najvjerojatnije je uzrokovana pojavom autoantitijela na eritropoetin i eritroblaste. Ova vrsta anemije obično se liječi kortikosteroidima iako se uspješno upotrebljavaju i ciklofosfamid i ciklosporin. U ovom radu prikazujemo slučaj bolesnice s PRCA-om u sistemskom eritemskom lupusu.