Xiong Nanqing, Gao Wen, Pan Junjie, Luo Xinping, Shi Haiming, Li Jian
Department of Cardiology, Huashan Hospital, Fudan University, 12 Wulumuqizhong Road, Shanghai, China.
J Thromb Thrombolysis. 2017 Jul;44(1):57-62. doi: 10.1007/s11239-017-1490-4.
For the patients with essential thrombocythemia (ET), systemic thrombosis presents as one of the most dangerous complications. It's been widely accepted that acute coronary syndrome (ACS) is a kind of thrombotic diseases. However, there are very few case reports about ET first presenting as ACS. For some patients diagnosed as ACS, but without markedly elevated platelet, underlying ET was missed. And there are some controversies in the principles and target of treatment in those patients. We reported three cases of ACS, in which the patients who did not have common risk factors for coronary artery diseases and presented only mild atherosclerotic stenosis during coronary angiography, one of which had recurrent coronary artery thrombosis. Noticing their elevated blood platelet level and characteristics in angiography, diagnosis of ET was made according to bone marrow morphology and genetic tests. Although they had only mild thrombocytosis, we applied intensive treatment with dual anti-platelet therapy combined with cytoreduction in addition to early coronary intervention, having satisfying outcomes. During the diagnosis and treatment of ACS, if patients present thrombocytosis, but lack common coronary disease risk factors and thrombotic coronary artery occlusion, cardiologists should search for possible ET as an underlying cause of thrombotic coronary event. All those patients were high-risk according to ET risk stratification. Treatment of cytoreduction in combination with anti-thrombosis therapy and revascularization are beneficial. Treatment aims at the target of complete response with platelet count below 400 × 10/L.
对于原发性血小板增多症（ET）患者，系统性血栓形成是最危险的并发症之一。急性冠状动脉综合征（ACS）是一种血栓性疾病，这一点已被广泛接受。然而，关于以ACS首次发病的ET病例报告却非常少。对于一些诊断为ACS但血小板无明显升高的患者，潜在的ET可能被漏诊。而且这些患者在治疗原则和目标方面存在一些争议。我们报告了3例ACS患者，这些患者没有常见的冠状动脉疾病危险因素，冠状动脉造影仅显示轻度动脉粥样硬化狭窄，其中1例发生复发性冠状动脉血栓形成。注意到他们的血小板水平升高及血管造影表现，根据骨髓形态学和基因检测确诊为ET。尽管他们仅有轻度血小板增多症，但除早期冠状动脉介入治疗外，我们采用双重抗血小板治疗联合细胞减灭的强化治疗，取得了满意的效果。在ACS的诊断和治疗过程中，如果患者出现血小板增多，但缺乏常见的冠状动脉疾病危险因素和血栓性冠状动脉闭塞，心脏病专家应寻找可能存在的ET作为血栓性冠状动脉事件的潜在病因。根据ET风险分层，所有这些患者均为高危患者。细胞减灭治疗联合抗血栓治疗和血运重建是有益的。治疗目标是使血小板计数低于400×10⁹/L并达到完全缓解。
