Onkológia 4/2015

Diagnostics and treatment of essential thrombocytemia – is there yet anything new?

Essential thrombocytemia (ET) is a myeloproliferative neoplasm (MPN) in whose diagnostics and treatment many novelties have occurred recently. In spite its course is relatively benign when compared with other MPN´s, patients are at any time in the course of the disease threatened with thromboembolic (TE) and/or haemorrhagic complications. TE event can be the first sign of the disease. The basic principle is to distinguish ET from secondary causes of thrombocytosis, which are much more frequent. After their exclusion a hematologist should perform examinations to exclude other MPN´s as well as molecular genetic tests to specify the molecular basis of ET. All these examinations should be done before the start of any therapy. Traditional drug hydroxyurea as a potential mutagen is reserved only for patients with cardio-vascular complications. Those are usually older patients, where the awaited life span is shorter than the time course in which mutagenic features of hydroxyurea can become evident. The drug of first choice in Slovakia is anagrelide – a non-cytostatic and non-mutagenic drug which acts at the level of megakaryocyte maturation. For younger patients in reproductive age also interferon-α is suitable in the case of good tolerance. Combination of anagrelide with hydroxyurea is also possible. It enables to administer lower and better tolerable doses of each drug. Treatment should be done according to modern – Central European – guidelines.

Keywords: essential thrombocytemia, myeloproliferative neoplasms/disorders, anagrelide, thrombophilia, thrombosis, genetic examination, bone marrow histology.