Introduction: In order to establish and use a national registry, several Hungarian hematology centers collected data of myeloproliferative neoplasia patients.
Aim: The recent publication is an analysis of the data of registered essential thrombocythaemic patients.
Method: an online electronic registry has been established, using 2008 World Health Organization's diagnostic criteria and thrombotic risk was evaluated according to Landolfi stratification.
Results: Data of 350 essential thrombocythaemic patients from 15 Hungarian hematology centers entered up to the date of June 30, 2015 were used for analysis. Patients were followed up to (median) 6 years. The epidemiologic data (age, gender) and thrombotic events prior and after the diagnosis, were similar to the literature. The thrombotic events of anagrelide treated patient (n = 139) and the hydroxyurea + aspirin treated patients (n = 141) have been compared. The major arterial and venous events were similar between the groups, but there were fivefold less minor arterial and venous events in the anagrelide group (p<0.001). Thrombotic incidence after diagnosis were influenced only by medication and thrombotic events before the diagnosis.
Conclusions: Anagrelide significantly decreased the number of patients experiencing minor arterial and minor venous thrombosis, vs hydroxyurea + aspirin. Despite of the treatment the risk of thrombotic events after diagnosis remained high, and was significantly increased in patients with thrombosis before diagnosis. Orv. Hetil., 2017, 158(3), 111-116.
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http://dx.doi.org/10.1556/650.2017.30638 | DOI Listing |
Orv Hetil
January 2017
Hematológiai Osztály, Somogy Megyei Kaposi Mór Oktató Kórház Kaposvár.
Introduction: In order to establish and use a national registry, several Hungarian hematology centers collected data of myeloproliferative neoplasia patients.
Aim: The recent publication is an analysis of the data of registered essential thrombocythaemic patients.
Method: an online electronic registry has been established, using 2008 World Health Organization's diagnostic criteria and thrombotic risk was evaluated according to Landolfi stratification.
Thromb Haemost
March 2012
Department of Plasma Proteins, Sanquin Research, Amsterdam, The Netherlands.
The natural anticoagulant protein S contains a so-called thrombin- sensitive region (TSR), which is susceptible to proteolytic cleavage. We have previously shown that a platelet-associated protease is able to cleave protein S under physiological plasma conditions in vitro . The aim of the present study was to investigate the relation between platelet-associated protein S cleaving activity and in vivo protein S cleavage, and to evaluate the impact of in vivo protein S cleavage on its anticoagulant activity.
View Article and Find Full Text PDFPlatelets
March 2004
Department of Haematology, Haemostasis Thrombosis Research, University Hospital Antwerp, Antwerp, Belgium.
Essential thrombocythaemia (ET) is associated with a broad spectrum of microvascular circulation disturbances including erythromelalgia and its ischaemic complications, episodic neurological symptoms of atypical and typical transient ischaemic attacks (TIAs), transient ocular ischaemic attacks, acute coronary syndromes, and superficial 'thrombophlebitis'. The microvascular circulation disturbances are caused by spontaneous activation and aggregation of hypersensitive thrombocythaemic platelets at high shear stress in the endarterial microcirculation involving the peripheral, cerebral and coronary circulation. As this microvascular syndrome is a pathognomonic feature of essential thrombocythaemia and of thrombocythaemia associated with polycythaemia vera (PV) in complete remission with normal haematocrit, we have labelled these two variants of thrombocythaemia as thrombocythaemia vera.
View Article and Find Full Text PDFBaillieres Clin Haematol
December 1998
Division of Hematology/Oncology, University of Alabama at Birmingham 35294, USA.
While significant progress has been made in understanding the cellular defect and molecular basis of polycythaemia vera (PV), elucidation of the primary mutation leading to PV remains elusive. While clinically useful, the PV diagnostic criteria put forward by the Polycythemia Vera Study Group are not based on the pathophysiology of this disorder and in some instances may lead to false diagnosis or may not be sufficient to diagnose an early PV. In diagnostically unclear situations, clinical and laboratory findings must take into account the acquired nature of PV, its clonality, and the presence of endogenous erythroid colony formation in serum-containing media.
View Article and Find Full Text PDFThromb Haemost
July 1997
Centro Ricerche Fisiopatologia dell'Emostasi, Università Cattolica del Sacro Cuore, Roma, Italy.
Arterial and venous thromboses and microcirculatory disturbances such as erythromelalgia and neurologic and visual symptoms are the thrombotic manifestations occurring in Polycythaemia Vera and Essential Thrombocythaemia. The increased in vivo thromboxane A2 generation existing in these patients and the selective sensitivity of erythromelalgia to aspirin suggest that platelet PGG/H synthase products may be involved in transducing the increased thrombotic risk. The relationship between Thromboxane A2 production and thrombotic accidents will be investigated by the European Collaboration on Low-Dose Aspirin in Polycythaemia Vera (ECLAP) which will test the efficacy of low-dose aspirin by a randomised clinical trial.
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