GUIDELINES FOR DIAGNOSTICS AND TREATMENT OF PRIMARY IMMUNE THROMBOCYTOPENIA IN ADULTS

Autori:

Silva Zupančić-Šalek, Dražen Pulanić, Slobodanka Ostojić-Kolonić, Vlatko Pejša, Toni Valković, Damir Nemet

Sažetak
Radna skupina za bolesti hemostaze i tromboze Hrvatske kooperativne grupe za hematološke bolesti (KROHEM), Referentni centar Ministarstva zdravlja Republike Hrvatske za nasljedne i stečene bolesti hemostaze te Hrvatsko hematološko društvo Hrvatskoga liječničkog zbora izradili su Hrvatske smjernice za dijagnostiku i liječenje odraslih bolesnika s primarnom imunosnom trombocitopenijom (ITP). Detaljnim kliničkim i laboratorijskim pregledom treba isključiti niz čestih uzroka sekundarne trombocitopenije. Predlaže se individualizirani pristup liječenju, gdje se odluka o početku aktivnog liječenja temelji na ozbiljnosti i riziku od krvarenja, broju trombocita, životnom stilu, dobi bolesnika i komor­biditetima. Prva linija terapije ITP-a jesu glukokortikoidi, s primjenom intravenskih imunoglobulina ili bez nje. U drugoj terapijskoj liniji predlaže se splenektomija ili liječenje agonistima trombopoetinskih receptora. Kod splenektomiranih ­bolesnika s relapsom ITP-a preporučuje se liječenje agonistima trombopoetinskih receptora. Alternativno se sugerira mogućnost primjene rituksimaba i drugih imunosupresiva u drugoj ili kasnijoj terapijskoj liniji.
Summary

Working Group for Diseases of Hemostasis and Thrombosis of the Croatian Cooperative Group for Hematologic Diseases (CROHEM), Referral Center for Hereditary and Acquired Disorders of Hemostasis of the Croatian Ministry of Health, and Croatian Hematology Society of the Croatian Medical Association developed the Croatian guidelines for the diagnosis and treatment of primary immune thrombocytopenia (ITP). Detailed clinical and laboratory examination should be performed to exclude common secondary causes of thrombocytopenia. An individualized therapeutic approach is ­proposed, and decision to start active treatment depends on the severity and risk of bleeding, platelet count, patient’s life style, age and comorbidities. Glucocorticoids with or without intravenous immunoglobulines are recommended as the first line treatment. The second line treatments are splenectomy or thrombopoietin receptor agonists as suggested options. Thrombopoietin receptor agonists are recommended treatment for splenectomized patients with relapsed ITP. Alternatively rituximab or other immunosuppressive agents are suggested in second or later treatment lines.