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原发性血小板增多症:大血管血栓形成的一个被低估的病因。

Essential thrombocytosis: underemphasized cause of large-vessel thrombosis.

作者信息

Johnson M, Gernsheimer T, Johansen K

机构信息

Department of Surgery, University of Washington School of Medicine, Seattle, USA.

出版信息

J Vasc Surg. 1995 Oct;22(4):443-7; discussion 448-9. doi: 10.1016/s0741-5214(95)70013-7.

Abstract

PURPOSE

The purpose of this study was to describe the clinical course of patients seen with large-vessel thrombosis in association with essential thrombocytosis (ET).

METHODS

This study was a retrospective review of all patients treated for large-vessel thrombosis caused by ET during a 2-year period at University of Washington teaching hospitals.

RESULTS

Five patients presented with arterial (femoral-popliteal-tibial: aortic), portal (two cases), or systemic venous (inferior vena cava) thrombosis and required operation. Two were known to have ET; in three others ET was diagnosed after operation when platelet counts persistently in excess of 500,000/mm3 were noted. The diagnosis of ET was established in each case by ruling out causes of reactive thrombocytosis and (in the three new cases) by evidence for megakaryocyte hyperplasia on bone marrow biopsy. Platelet counts in all five patients were reduced to normal levels by cytoreductive therapy, and no further thrombotic episodes have occurred during 18 months (mean) of follow-up. During this 2-year period ET accounted for more large-vessel thrombotic complications in our institutions than all other more frequently described hypercoagulable states combined.

CONCLUSIONS

ET is an underemphasized cause of large-vessel thrombosis.

摘要

目的

本研究旨在描述伴有原发性血小板增多症(ET)的大血管血栓形成患者的临床病程。

方法

本研究是对华盛顿大学教学医院2年内因ET导致大血管血栓形成而接受治疗的所有患者进行的回顾性研究。

结果

5例患者出现动脉(股-腘-胫:主动脉)、门静脉(2例)或体静脉(下腔静脉)血栓形成并需要手术治疗。其中2例已知患有ET;另外3例在术后血小板计数持续超过500,000/mm³时被诊断为ET。通过排除反应性血小板增多症的病因(在3例新病例中)以及骨髓活检显示巨核细胞增生的证据,确诊了每例ET。所有5例患者的血小板计数通过细胞减灭疗法降至正常水平,并且在18个月(平均)的随访期间未发生进一步的血栓形成事件。在这2年期间,ET在我们机构中导致的大血管血栓形成并发症比所有其他更常描述的高凝状态加起来还要多。

结论

ET是大血管血栓形成一个未得到充分重视的病因。

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