ANAGRELIDE FOR TREATMENT OF PATIENTS WITH ESSENTIAL THROMBOCYTHAEMIA

Autori:

Ana Boban, Dubravka Sertić, Ivo Radman, Silva Zupančić-Šalek, Renata Zadro, Boris Labar

Sažetak

Sažetak. Anagrelid je derivat imidazokinazolina, a smanjuje broj trombocita djelujući inhibitorno na proliferaciju i sazrijevanje megakariocita. Budući da ne djeluje citostatski, već selektivno pogađa trombocitnu lozu, hematološke nuspojave poput anemije i leukopenije viđaju se vrlo rijetko. Zbog ovih je obilježja pogodan u liječenju kroničnih mijeloproliferativnih bolesti u kojima dominira trombocitemija. U ovom smo radu pokazali djelotvornost anagrelida kao druge linije liječenja u 14 bolesnika s postavljenom dijagnozom esencijalne trombocitemije. Dobar odgovor na terapiju postiglo je 11 bolesnika (78%). Kriterij odgovora bio je broj trombocita manji od 450×109/l odnosno 700×109/l uz odsutnost trombohemoragijskih događaja. Terapija je prekinuta u 6 bolesnika; jedan bolesnik zbog razvoja ozbiljne nuspojave, troje zbog refrakternosti na terapiju, jedna bolesnica na vlastiti zahtjev te jedna bolesnica zbog planiranja trudnoće. Zaključili smo da je anagrelid učinkovit i siguran lijek u drugoj liniji liječenja bolesnika s esencijalnom trombocitemijom.

Summary

Summary. Anagrelide is an imidazokinazoline derivate that reduces platelet production by interfering with megakaryocyte proliferation and maturation. As a non-cytostatic drug it selectively affects megakaryocyte lineage and therefore anemia and leukocytopenia are not likely to occur. This makes anagrelide adequate for the treatment of chronic myeloproliferative disorders characterized by marked thrombocytemia. In this study we have evaluated efficacy of anagrelide in 14 pretreated patients with essential thrombocytemia. The response was achieved in 11 patients (78%) and was defined as a platelet count lower than 450× 109/l or 700×109/l without thrombohemorrhagic incidents. The therapy was stopped in 6 patients; three patients did not respond to treatment; one had a serious side effect; pregnancy was the reason for discontinuation of therapy in one patient, and in one patient therapy was changed by his own request. We can conclude that anagrelide is an effective and safe drug for pretreated patients with essential thrombocythemia.