Thrombocytosis and thrombocythemia

Česká verzia

Authors: J. Schwarz ¹; M. Penka ²
Authors place of work: Klinický úsek Ústavu hematologie a krevní transfuze, Praha, přednosta doc. MUDr. Petr Cetkovský, Ph. D. ¹; Oddělení klinické hematologie FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Miroslav Penka, CSc. ²
Published in the journal: Vnitř Lék 2005; 91(7 a 8): 861-872
Category: 128th Internal Medicine Day - 21rd Vanysek's Day Brno 2005

Summary

In this review, we recommend the nosological classification of the Ph- myeloproliferative disorders with thrombocythemia (MPD-T) to be performed either according to the WHO or to the Rotterdam criteria, both of which are based on demonstration of specific histopathological features of the respective MPD-T. The most frequent of the primary MPD-Ts is the proliferative, prefibrotic or early fibrotic phase of idiopathic myelofibrosis, but reactive thrombocytoses are even more frequently seen. The differences between the primary MPD-Ts and secondary thrombocytoses are discussed along with the issue of clonality of these disorders and the methodology of its determination. The most serious complications of MPD-Ts are the following: 1. arterial and venous thromboses, 2. transformation of MPD-T into secondary acute myeloid leukemia, 3. microcirculation problems, 4. hemorrhage. The main risk factors of thrombosis are age, history of previous thrombosis, thrombocyte counts 350-2200 x 109/L and the presence of an additional thrombophilic risk factor (hereditary thrombophilia, malignancy, gravidity, hypercoagulable states, cardiovascular disease). The treatment of MPD-Ts, according to the prepared recommendations of the Czech multiinstitutional group, should be stratified with respect to the thrombotic and hemorrhagic risks. In high-risk patients, thromboreductive treatment is warranted. Seeing to the possible leukemogenicity of all the cytostatic drugs used, including hydroxyurea, they should not be administered over prolonged periods of time to individuals less than 60 years old. In these patients, interferon-α or anagrelide should be preferred. In low-risk patients, antiaggregation therapy is sufficient.

Key words:
myeloproliferative disease - thrombocythemia - secondary thrombocytosis - risk factors - diagnostics - clonality - leukemic transformation - thrombosis - treatment algorithm - hydroxyurea - interferon - anagrelide

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