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Summary. Background: Little information is available on the long-term clinical outcome of cerebral vein thrombosis (CVT). Objectives and methods:In an international, retrospective cohort study, we assessed the long-term rates of mortality, residual disability and recurrent venous thromboembolism (VTE) in a cohort of patients with a first CVT episode. Results: Seven hundred and six patients (73.7% females) with CVT were included. Patients were followed for a total of 3171 patient-years. Median follow-up was 40 months (range 6, 297 months). At the end of follow-up, 20 patients had died (2.8%). The outcome was generally good: 89.1% of patients had a complete recovery (modified Rankin Score [mRS] 0-1) and 3.8% had a partial recovery and were independent (mRS 2). Eighty-four per cent of patients were treated with oral anticoagulants and the mean treatment duration was 12 months. CVT recurred in 31 patients (4.4%), and 46 patients (6.5%) had a VTE in a different site, for an overall incidence of recurrence of 23.6 events per 1000 patient-years (95% confidence Interval [CI] 17.8, 28.7) and of 35.1 events/1000 patientyears (95% CI, 27.7, 44.4) after anticoagulant therapy withdrawal. A previous VTE was the only significant predictor of recurrence at multivariate analysis (hazard ratio [HR] 2.70; 95% CI 1.25, 5.83). Conclusions: The long-term risk of mortality and recurrent VTE appears to be low in patients who survived the acute phase of CVT. A previous VTE history independently predicts recurrent events.

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Controversies still exist regarding definition of the thrombotic risks in Ph-(BCR/ABL1-) myeloproliferative disorders with thrombocythemia (MPD-T). Platelet counts at diagnosis are currently not taken as a risk factor of thrombosis. In our cohort of 1179 patients with MPD-T, prospectively registered for anagrelide treatment, we found that the median platelet count prior to the thrombotic event was significantly higher than at time points without any ensuing thrombosis (453 vs. 400 9 10 9 /L, P < 0.001), albeit higher platelet counts at diagnosis tended to be connected with fewer thrombotic events (in contrast to WBC counts at diagnosis). The JAK2 V617F mutation predicted both arterial and venous events, while age >65 yr, hypertension, diabetes mellitus, smoking, elevated triglyceride and homocysteine levels predicted arterial events only. For venous events, the specific thrombophilic risk factors (factor V 'Leiden' and others), antiphospholipid antibodies, and elevated factor VIII levels played a major role. During anagrelide treatment (AE aspirin), we documented a decrease in both venous (6.7-fold) and arterial events (1.8-fold), while bleeding (mostly minor events) increased twofold compared to history. Our results suggest that keeping platelet counts at low levels may be a meaningful therapeutic measure to prevent thrombosis, although their counts at diagnosis lack any prognostic value.

Background and purpose of the workshopConcern about the venous thromboembolism (VTE) risk of new hormonal contraceptive options shortly after their entry into the market has triggered a number of 'pill scares', each of which resulted in panic stopping of the formulations in question and a spike in unplanned pregnancies, yet with no subsequent reduction in VTE rates among women of reproductive age.Perhaps the best example of a recent pill scare that resulted in enormous harm from a public health perspective was the 'third-generation pill scare' that occurred in many countries in Europe and around the world in 1995. At that time the new third-generation pills were promoted as being less androgenic and as possibly having fewer adverse effects on cardiovascular and metabolic parameters and thereby potentially being safer than existing pills.Shortly after the introduction of these third-generation pills, reports of a possible increased risk of VTE began to appear. Such reports brought the progestogen component of these pills under scrutiny and inevitably a phenomenon known as 'stimulated reporting' occurred. Physicians with patients on these new pills were more likely to send their patients for assessment of any leg pain or swelling and more likely to report any VTE episodes to regulatory authorities because of heightened awareness of the possible risk. What followed was an international pill scare when regulatory authorities in a number of countries issued alerts about the possible increased risk of VTE with thirdgeneration pills. Panic stopping of pills by millions of women resulted in an abrupt and alarming rise in unplanned pregnancy as evidenced by sudden increases in deliveries and abortions, 1-3 each with their attendant increased risks of VTE.The history of this unfortunate episode is well documented. After class action lawsuits and lengthy trials, at which experts in epidemiology debated the findings, there emerged an awareness that studies examining VTE risks, in particular, required an understanding of the epidemiology of VTE and the possible biases that might systematically result in findings which diverged from the 'truth'. 4,5 The precise effects of different hormonal contraceptives on the haemostatic system continue to be studied and debated 6,7 but because compelling data for an increased risk have not been demonstrated the lawsuits were ultimately thrown out 8 and third-generation pills remain on the market and are widely prescribed today. 9 In 2009, anecdotal reports of VTE episodes in women using an ethinylestradiol/drospirenone (EE/DRSP)-based oral contraceptive (Yasmin ® ) were followed by publications with conflicting findings about the risk of VTE with this product. This led the manufacturer to invite experts in gynaecology, reproductive endocrinology, haematology and epidemiology to a workshop to critically review these recent publications to understand the reasons for divergent results and to produce a consensus statement about the likely comparative risk of this new formulation and other mar...