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慢性骨髓增殖性疾病中的出血与血栓形成:血小板疾病与疾病临床特征的关系

Bleeding and thrombosis in chronic myeloproliferative disorders: relation of platelet disorders to clinical aspects of the disease.

作者信息

Wehmeier A, Scharf R E, Fricke S, Schneider W

机构信息

Department of Internal Medicine, University of Düsseldorf, FRG.

出版信息

Haemostasis. 1989;19(5):251-9. doi: 10.1159/000215980.

Abstract

Bleeding and thrombosis are a major cause of morbidity and mortality in myeloproliferative disorders (MPD). This study evaluates the relation between thrombohemorrhagic complications and platelet abnormalities in different subgroups of MPD. In 57 MPD patients thrombohemorrhagic complications occurred in 71% of patients with polycythemia rubra vera and 50% of patients with osteomyelofibrosis and primary thrombocythemia but in only 29% of patients with chronic myelogenous leukemia. Increased beta-thromboglobulin and platelet factor 4 plasma levels, platelet aggregation defects, and increased dispersion of the platelet volume distribution curve were most frequent in those subgroups where most serious thrombohemorrhagic complications were observed, and multiple platelet-related abnormalities were often found simultaneously. Fibrinopeptide A plasma levels were rarely elevated, however. Our results indicate that platelet abnormalities associated with bleeding and thrombosis are primarily determined by the clinical subgroup of myeloproliferative disease.

摘要

出血和血栓形成是骨髓增殖性疾病(MPD)发病和死亡的主要原因。本研究评估了MPD不同亚组中血栓出血并发症与血小板异常之间的关系。在57例MPD患者中,血栓出血并发症发生在71%的真性红细胞增多症患者、50%的骨髓纤维化和原发性血小板增多症患者中,但仅发生在29%的慢性粒细胞白血病患者中。β-血小板球蛋白和血小板因子4血浆水平升高、血小板聚集缺陷以及血小板体积分布曲线离散度增加在观察到最严重血栓出血并发症的亚组中最为常见,并且经常同时发现多种与血小板相关的异常。然而,纤维蛋白肽A血浆水平很少升高。我们的结果表明,与出血和血栓形成相关的血小板异常主要由骨髓增殖性疾病的临床亚组决定。

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