Essential thrombocythaemia and other myeloproliferative disorders with thrombocythaemia treated with Thromboreductin. A report from the database of Register for the 1st quarter of 2010


Authors: M. Penka 1;  J. Schwarz 2;  P. Ovesná 3;  A. Hluší 4;  Z. Kořístek 5;  M. Doubek 5;  P. Ďulíček 6;  D. Pospíšilová 7;  J. Kissová 1;  A. Buliková 1;  T. Pavlík 3;  Kolektiv České Pracovní Skupiny Pro Myeloproliferativní Choroby (czemp)
Authors‘ workplace: Oddělení klinické hematologie FN Brno, pracoviště Bohunice, přednosta prof. MU Dr. Miroslav Penka, CSc. 2Ústav hematologie a krevní transfuze Praha, ředitel prof. MU Dr. Marek Trněný, CSc. 3Institut bio­statiky a analýz Lékařské a Přírodovědecké fakulty M 1
Published in: Vnitř Lék 2010; 56(6): 503-512
Category: Original Contributions

Overview

In the Czech Republic, anagrelid is used according to the recommendations of the Czech Working Group on Myeloproliferative Disorders for treatment of thrombocythaemias associated with chronic myeloproliferative disorders –  mainly essential thrombocythaemia and, regularly, reports are being presented from the Register of Patients Treated with Thromboreductin®, most recently last year (Vnitř Lék 2009; 55: I– XII). The Register commenced in 2005 and from then it aims to determine detailed clinical and laboratory profiles of the patients. The structure of the Register has changed significantly in the course of its existence, reflecting the reports from each of the analyses conducted so far. Also, the data entry in the database improves every year and it reaches 97% on some of the items. The longest evaluation period in some of the patients is 108 months. By April 2010, the Register database contained data on 717 patients. Of these, 672 patients with the diagnosis of a Ph‑ negative chronic myeloproliferative disorder were evaluated. This year’s analysis included the patients with essential thrombocythaemia, polycythaemia vera and primary myelofibrosis only. The analysis included 418 women and 254 men with median age of 50 years. Unlike the first years, 2/ 3 of the current sample are non pretreated patients, meaning that the patients reach the specialized centres early in their treatment. Also, patients, and the older patients in particular, are more frequently treated with combined regimens including Thromboreductin®. We increasingly observe hypertension as one of the monitored risk factors preceding the disease and laboratory parameters show JAK2 mutation in more than a half of patients while some form of thrombotic diathesis is found in the anamnesis of 7– 10% of patients. Some bleeding is observed in 1– 5% of the registered patients. In comparison to the previous years, this is a decrease in the prevalence of clinical symptoms prior to the disease onset; this is very likely associated with an earlier patient diagnosis within the asymptomatic phase of the disease. Therapeutically, we achieve a fast treatment response but there still are 16.3% of sufficient after one year of treatment. Thromboreductin® dose is increasing but even in this group it does not exceeds the mean of 2.38 mg per 24 hours. Complications are observed in 6.2% of patients in the first year of therapy, and of these, thrombotic events in about 2.5% and (small) bleeding complications in 4% of patients. The data suggest that we still do not reach treatment response in a certain proportion of patients after a year of their therapy. Even though the care results from the analysed data improve every year, the Register helps to uncover some issues that still remain, such as treatment intensification and other treatment modifications.

Key words:
essential thrombocytopenia –  myeloproliferative disorders –  anagrelid (Thromboreductin®) –  Register –  JAK2 mutation –  thrombophilia


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