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各种骨髓增殖性疾病血小板增多症中的非典型短暂性脑缺血发作

Atypical transient ischemic attacks in thrombocythemia of various myeloproliferative disorders.

作者信息

Michiels J J, van Genderen P J, Jansen P H, Koudstaal P J

机构信息

Department of Hematology, University Hospital Dijkzigt, Erasmus University Medical School, Rotterdam, The Netherlands.

出版信息

Leuk Lymphoma. 1996 Sep;22 Suppl 1:65-70. doi: 10.3109/10428199609074362.

DOI:10.3109/10428199609074362
PMID:8951774
Abstract

Neurological symptoms of transient unsteadiness, dysarthria, dysphasia, dysbasia, transient monoor hemiparesis, hemiparesis, scintillating scotomas, amaurosis fugax, vertigo, dizziness, migraine accompaniments, syncope and seizures were the presenting manifestations of thrombocythemia in various myeloproliferative disorders. Erythromelalgia preceded or followed the neurologic ischemic attacks. The neurologic and ocular attacks usually had a sudden onset, lasted for a few seconds to several minutes and occurred independently or sequentially rather than simultaneously. This clinical syndrome is caused by platelet-mediated ischemic and thrombotic processes in the end-arterial microvasculature and reflects the existence of a platelet dependent and aspirin responsive arterial thrombophilia in thrombocythemia as novel disease entity, which confirms and elucidates Mitchell's hypothesis.

摘要

短暂性步态不稳、构音障碍、言语困难、共济失调、短暂性单瘫或偏瘫、偏瘫、闪光暗点、一过性黑矇、眩晕、头晕、偏头痛伴随症状、晕厥和癫痫发作等神经系统症状是各种骨髓增殖性疾病中血小板增多症的主要表现。红斑性肢痛症先于或后于神经缺血性发作出现。神经和眼部发作通常起病突然,持续数秒至数分钟,可独立发生或相继出现,而非同时发生。这种临床综合征是由终末动脉微血管系统中血小板介导的缺血和血栓形成过程引起的,反映了血小板增多症中一种新的疾病实体——血小板依赖性且对阿司匹林有反应的动脉血栓形成倾向的存在,这证实并阐明了米切尔的假说。

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