Aspirin responsive platelet thrombophilia in essential thrombocythemia and polycythemia vera

• 作者: Fibo WJ Ten Kate Jan Jacques Michiels Perry JJ Van Genderen Peter J Koudstaal
• 来源期刊: 世界血液学杂志
• 学科: 医学
• 关键词: Erythromelalgia Migraine-like cerebral transient ischemic attacks Platelets β-thromboglobulin Thrombomoduline Thrombosis ASPIRIN ANTICOAGULATION Arterial PLATELET THROMBOPHILIA Essential THROMBOCYTHEMIA POLYCYTHEMIA vera
• 年，卷（期）: 2013,（2）
• 页码范围: 20-43
• 页数: 24页
• 分类号: R

摘要

Essential thrombocythemia(ET)and polycythemia vera(PV)frequently present with erythromelalgia and acrocyanotic complications,migraine-like microvascular cerebral and ocular transient ischemic attacks(MIAs)and/or acute coronary disease.The spectrum of MIAs in ET range from poorly localized symptoms of transient unsteadiness,dysarthria and scintillating scotoma to focal symptoms of transient monocular blindness,transient mono-or hemiparesis or both.The attacks all have a sudden onset,occur sequentially rather than simultaneously,last for a few seconds to several minutes and are usually associated with a dull,pulsatile or migraine-like headache.Increased hematocrit and blood viscosity in PV patients aggravate the microvascular ischemic syndrome of thrombocythemia to major arterial and venous thrombotic complications.Phlebotomy to correct hematocrit to normal in PV significantly reduces major arterial and venous thrombotic complications,but fails to prevent the platelet-mediated erythromelalgia and MIAs.Complete long-term relief of the erythromelalgic microvascular disturbances,MIAs and major thrombosis in ET and PV patients can be obtained with low dose aspirin and platelet reduction to normal,but not with anticoagulation.Skin punch biopsies from the erythromelalgic area show fibromuscular intimal proliferation of arterioles complicated by occlusive plateletrich thrombi leading to acrocyanotic ischemia.Symptomatic ET patients with erythromelalgic microvascular disturbances have shortened platelet survival,increased platelet activation markersβ-thromboglobulin(β-TG),platelet factor 4(PF4)and thrombomoduline(TM),increased urinary thromboxane B2(TXB2)excretion,and no activation of the coagulation markers thrombin fragments F1+2 and fibrin degradation products.Inhibition of platelet cyclooxygenase(COX1)by aspirin is followed by the disappearance and no recurrence of microvascular disturbances,increase in platelet number,correction of the shortened platelet survival times to normal,and reduction of increased plasma lev