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^®

Česká verzia

Authors: M. Penka ¹; J. Schwarz ²; T. Pavlík ³; R. Pytlík ⁴; M. Do ubek ⁵; P. Ďulíček ⁶; J. Kissová ¹; A. Hluší ⁷; M. Schutzova ⁸; O. Černá ⁹; Y. Brychtová ¹; T. Szotkowski ⁷; Z. Volková ³; J. Seghetová ¹⁰; V. Vozobulová ⁸; I. Hadačová ¹⁰; I. Hochová ¹⁰; J. Voglová ⁶; L. Dušek ³
Authors place of work: Oddělení klinické hematologi e FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Miroslav Penka, CSc. ¹; Klinický úsek Ústavu hematologi e a krevní transfuze Praha, přednosta doc. MUDr. Petr Cetkovský, Ph. D. ²; Institut bi ostatistiky a analýz MU Brno, přednosta doc. RNDr. Ladislav Dušek, CSc. ³; I. interní klinika 1. lékařské fakulty UK a VFN Praha, přednosta prof. MUDr. Pavel Klener, DrSc. ⁴; Interní hemato onkologická klinika LF MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Jiří Vorlíček, CSc. ⁵; Oddělení klinické hematologi e II. interní kliniky LF UK a FN Hradec Králové, přednosta prof. MUDr. Jaroslav Malý, CSc. ⁶; Hemato- onkologická klinika Lékařské fakulty UP a FN Olomo uc, přednosta prof. MUDr. Karel Indrák, DrSc. ⁷; Hematologicko- onkologické oddělení FN Plzeň, přednosta prim. MUDr. Vladimír Koza ⁸; Oddělení klinické hematologi e FN Královské Vinohrady Praha, přednosta doc. MUDr. Tomáš Kozák, Ph. D. ⁹; Oddělení klinické hematologi e FN Motol, Praha, přednostka prim. MUDr. Ivana Hochová ¹⁰
Published in the journal: Vnitř Lék 2008; 54(7-8): 775-782
Category: Original Contributions

Summary

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 thrombocythemia. 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.

Key words:
essential thrombocythemia – myeloproliferations – anagrelid (Thromboreductine^®) – registry – JAK2 mutation – thrombophilia

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