Radaelli Franca, Colombi Mariangela, Calori Rossella, Zilioli Vittorio Ruggero, Bramanti Stefania, Iurlo Alessandra, Zanella Alberto
Unità Operativa Ematologia 2, Fondazione IRCCS Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, Milan, Italy.
Hematol Oncol. 2007 Sep;25(3):115-20. doi: 10.1002/hon.816.
Thrombotic and haemorrhagic complications are the main causes of morbidity in Essential Thrombocythemia (ET). We investigated the clinical and laboratory characteristics associated with the occurrence of these events with the aim of identifying subgroups of patients who might benefit from anti-aggregant and/or cytoreductive therapy. The study involved 306 consecutive ET patients (median age 58 years and median follow-up 96 months); the investigated variables were age, gender, platelet count, previous history of thrombotic or haemorrhagic events, disease duration and cardiovascular risk factors. Forty-six patients (15%) experienced thrombotic complications during the follow-up: 26/64 patients with a previous history of thrombosis (40.6%) and 20/242 patients without (8.3%; p < 0.0001). Thirty-one patients (10%) experienced major haemorrhagic complications, mainly gastrointestinal tract bleeding: 3 with and 28 without a history of haemorrhagic events (p = 0.052). When the patients with a negative history of thrombosis were stratified on the basis of the number of cardiovascular risk factors (none vs. one vs. more than one), there was a significant correlation with the occurrence of thrombotic events (p < 0.05). ET patients with a positive history of thrombosis are at high risk of thrombotic complications, and should receive cytoreductive and anti-aggregant treatment. Asymptomatic patients with a negative thrombotic history and no cardiovascular risk factors are at low risk, and should not be treated. Patients with a negative thrombotic history and one or more cardiovascular risk factors are at intermediate risk, and should be treated with anti-aggregant and/or cytoreductive therapy. The need for treatment should be periodically re-evaluated. Age and platelet count, generally accepted as very important risk factors for thrombosis, did not seem in our series associated with an increased risk for thrombosis.
血栓形成和出血并发症是原发性血小板增多症(ET)发病的主要原因。我们调查了与这些事件发生相关的临床和实验室特征,旨在识别可能从抗聚集和/或减细胞治疗中获益的患者亚组。该研究纳入了306例连续的ET患者(中位年龄58岁,中位随访96个月);所研究的变量包括年龄、性别、血小板计数、既往血栓形成或出血事件史、疾病持续时间和心血管危险因素。46例患者(15%)在随访期间发生血栓形成并发症:64例有既往血栓形成史的患者中有26例(40.6%),242例无既往血栓形成史的患者中有20例(8.3%;p<0.0001)。31例患者(10%)发生严重出血并发症,主要为胃肠道出血:有出血事件史的3例和无出血事件史的28例(p = 0.052)。当无血栓形成史的患者根据心血管危险因素的数量进行分层(无 vs. 1个 vs. 超过1个)时,与血栓形成事件的发生存在显著相关性(p<0.05)。有血栓形成史阳性的ET患者发生血栓形成并发症的风险高,应接受减细胞和抗聚集治疗。无血栓形成史且无心血管危险因素的无症状患者风险低,不应接受治疗。无血栓形成史且有1个或更多心血管危险因素的患者风险中等,应接受抗聚集和/或减细胞治疗。治疗需求应定期重新评估。年龄和血小板计数通常被认为是血栓形成非常重要的危险因素,但在我们的系列研究中似乎与血栓形成风险增加无关。