Jabaily J, Iland H J, Laszlo J, Massey E W, Faguet G B, Brière J, Landaw S A, Pisciotta A V
Ann Intern Med. 1983 Oct;99(4):513-8. doi: 10.7326/0003-4819-99-4-513.
Essential thrombocythemia is a clonal myeloproliferative disorder, characterized predominantly by a markedly elevated platelet count without known cause. We report a case that was recognized during investigation of a transient ischemic attack, and review the neurologic findings in 33 patients with unequivocal essential thrombocythemia under prospective study by the Polycythemia Vera Study Group. Twenty-one patients had neurologic manifestations at some point during their course, including headache (13 patients), paresthesiae (10), posterior cerebral circulatory ischemia (9), anterior cerebral circulatory ischemia (6), visual disturbances (6) and epileptic seizures (2). All patients with neurologic symptoms responded satisfactorily to treatment, although continuous or repeated treatment was often required. Therapeutic recommendations include plateletpheresis for major thrombo-hemorrhagic phenomena, or megakaryocyte suppression with radioactive phosphorus, alkylating agents (such as melphalan), or hydroxyurea; minor symptoms may respond to platelet antiaggregating agents.
原发性血小板增多症是一种克隆性骨髓增殖性疾病，主要特征为血小板计数显著升高且病因不明。我们报告了1例在短暂性脑缺血发作调查期间确诊的病例，并回顾了真性红细胞增多症研究组前瞻性研究的33例明确诊断为原发性血小板增多症患者的神经学表现。21例患者在病程中的某些时候出现神经学表现，包括头痛（13例）、感觉异常（10例）、大脑后循环缺血（9例）、大脑前循环缺血（6例）、视觉障碍（6例）和癫痫发作（2例）。所有有神经症状的患者对治疗反应良好，尽管常常需要持续或重复治疗。治疗建议包括对主要血栓出血现象进行血小板单采术，或用放射性磷、烷化剂（如美法仑）或羟基脲抑制巨核细胞；轻微症状可能对血小板抗聚集剂有反应。
