Aspirin responsive platelet thrombophilia in essential thrombocythemia and polycythemia vera
Aspirin responsive platelet thrombophilia in essential thrombocythemia and polycythemia vera作者机构：Department of Medicine Erasmus Medical Center Erasmus University Rotterdam 3015 GE RotterdamThe Netherlands Department of Hematology University Hospital Antwerp B-2650 Edgem Antwerp Belgium European Working Group on Myelopro-liferative Neoplasms Goodheart Institute and Foundation 3069 AT Rotterdam The Netherlands Department of Pathology Erasmus Medical Center Erasmus University Rotterdam 3015 GE Rotterdam The Netherlands Department of Neurology Erasmus Medical Center Rotterdam Erasmus University Rotterdam 3015 GE Rotterdam The Netherlands Department of Internal Medicine Haven Hospital 3011 TD RotterdamThe Netherlands Erasmus Medical Center Rotterdam3015 GE Rotterdam The Netherlands
出 版 物：《World Journal of Hematology》 (世界血液学杂志)
年 卷 期：2013年第2卷第2期
页 面：20-43页
学科分类：10[医学]
主 题：Erythromelalgia Migraine-like cerebral transient ischemic attacks Platelets β-thromboglobulin Thrombomoduline Thrombosis Aspirin Anticoagulation Arterial platelet thrombophilia Essential thrombocythemia Polycythemia vera
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 markers β-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 shor
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