DIAGNOSIS AND THERAPY FOR PATIENTS WITH ESSENTIAL THROMBOCYTHEMIA Guidelines of Croatian Cooperative Group for hematologic disorders – KROHEM
Autori:
Boris Labar, Rajko Kušec, Branimir Jakšić, Ljubica Škare-Librenjak, Antica Načinović-Duletić, Jasminka Petričević-Sinčić, Dubravka Čaržavec, Božena Coha, Velka Gverić-Krečak, Elizabeta Čorović, Nada Lang, Zlata Lojen-Nemet, Renata Babok-Flegarić
Sažetak
Sažetak. Esencijalna trombocitopenija (ET) klonski je mijeloproliferativni zloćudni tumor. Hrvatska kooperativna grupa za hematološke bolesti, KROHEM, predlaže smjernice dijagnostičkog i terapijskog pristupa. Dijagnoza ET temelji se na kriterijima dijagnostike i podjele mijeloidnih zloćudnih tumora Svjetske zdravstvene organizacije (SZO). Razina terapijskih preporuka polazi od pokazatelja predloženih od elektronskog izvora medicinskih informacija, UpToDate®. Za dijagnozu ET potrebno je dokazati trajan porast broja trombocita (>450×109/L), uz tipičnu histološku sliku megakariocitopoeze u koštanoj srži te isključiti postojanje sekundarne trombocitoze ili drugih kroničnih mijeloproliferativnih zloćudnih tumora. Liječenje se temelji na pokazateljima rizika od bolesti koji je određen brojen trombocita, dobi bolesnika te prisutnošću rizika ili znakova tromboze i krvarenja. Bolesnike niskog rizika (dob 60 godina) prva linija terapije je hidroksiureja, a anagrelid se primjenjuje u bolesnika s nepotpunim odgovorom na hidroksiureju. U trudnica, kada je potrebno liječenje, preporučuje se alfa-interferon.
Summary
Summary. Essential thrombocythemia (ET) is a clonal myeloproliferative neoplasm. Croatian Cooperative Group for hematologic disorders, KROHEM proposes the diagnostic and treatment guidelines for ET. Diagnosis of ET is based on the criteria and classification of World Health Organization (WHO). The level of treatment recommendation is based on the UpToDate® (web based medical community database) criteria. For ET diagnosis it is mandatory to show sustained increased number of platelets with typical histomorphological changes of megakaryopoiesis in bone marrow. Secondaly thrombocytosis and other chronic myeloproliferative neoplasms have to be excluded. Therapy is based on risk factors for ET. The risk factors are number of platelets, patient’s age, and the risk levels for thrombosis and bleeding. Patients with low risk (age <60 years and platelets <1000×109/L) arw not candidates for therapy. In younger group of patients with platelets between 1000 and 1500×109/L or more than 1500×109/L treatment with anagrelide or hydroxyurea is recommended respectively. In high risk patients hydroxyurea is the first line treatment. Anagrelide is indicated in these patients in the absence of treatment response. Alpha-interferon is recommended for pregnant women with ET and high platelet counts.
