Vnitr Lek 2008, 54(7-8):775-782

What is the current treatment of patients with essential thrombocytopenia and other myeloproliferations accompanied with thrombocytopenia, and what can be the predictive sign of the risk of thrombosis in such patients - a report from the registry of patients treated by Thromboreductine®

M. Penka^1,*, J. Schwarz², T. Pavlík³, R. Pytlík⁴, M. Doubek⁵, P. Dulíček⁶, J. Kissová¹, A. Hluší⁷, M. Schützová⁸, O. Černá⁹, Y. Brychtová¹, T. Szotkowski⁷, Z. Volková³, J. Seghetová¹⁰, V. Vozobulová⁸, I. Hadačová¹⁰, I. Hochová¹⁰, J. Voglová⁶, L. Dušek³

¹ Oddělení klinické hematologie FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Miroslav Penka, CSc.

² Klinický úsek Ústavu hematologie a krevní transfuze Praha, přednosta doc. MUDr. Petr Cetkovský, Ph.D.

³ Institut biostatistiky a analýz MU Brno, přednosta doc. RNDr. Ladislav Dušek, CSc.

⁴ I. interní klinika 1. lékařské fakulty UK a VFN Praha, přednosta prof. MUDr. Pavel Klener, DrSc.

⁵ Interní hematoonkologická klinika LF MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Jiří Vorlíček, CSc.

⁶ Oddělení klinické hematologie II. interní kliniky LF UK a FN Hradec Králové, přednosta prof. MUDr. Jaroslav Malý, CSc.

⁷ Hemato-onkologická klinika Lékařské fakulty UP a FN Olomouc, přednosta prof. MUDr. Karel Indrák, DrSc.

⁸ Hematologicko-onkologické oddělení FN Plzeň, přednosta prim. MUDr. Vladimír Koza

⁹ Oddělení klinické hematologie FN Královské Vinohrady Praha, přednosta doc. MUDr. Tomáš Kozák, Ph.D.

¹⁰ Oddělení klinické hematologie FN Motol, Praha, přednostka prim. MUDr. Ivana Hochová

The registry of patients treated with Thromboreductine® (anagrelid) in the contributing centres in the Czech Republic has been updated with data on the patients receiving this medication since 2004. The original purpose of the registry was to record responses to Thromboreductine® therapy and adverse drug reactions in patients with essential thrombocytopenia. However, data on additional Ph negative myeloproliferations, as well as data on cytoreductive therapies other than exclusively that using Thromboreductine® has also been recorded in the course of its compilation, including data on combined regimes. At present, the database contains data on 421 patients, and valid conclusions can be drawn if the level of data filling is enhanced. Evaluation has been currently focused on the analysis of the risk of development of clinical symptoms of thrombosis and on the standards of treatment from the viewpoint of the achieved treatment response. Analyses of data from the registry corroborate the special importance of the proof of JAK2 mutation, and of the test for factor V Leiden mutation, and of protein of S for the assessment of the risk of thromboembolic complications. The output of the analysis confirms that anagrelid is a very efficient thromboreductive agent the administration of which is associated with a low incidence of non-serious adverse effects (10.9%). However, in spite of a fast response to therapy, the therapeutic goal consisting in the reduction of the platelet count below 400 (or below 600) × 10⁹/l, i.e. the complete (or partial) treatment response, is relatively slow to achieve. This is likely to be due to lack of radical corrections in the dosage of the drug for different reasons.

Keywords: essential thrombocytopenia; myeloproliferations; anagrelid (Thromboreductine®); registry; JAK2 mutation; thrombophilia

Received: July 5, 2008; Published: July 1, 2008  Show citation