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与单克隆丙种球蛋白病相关的止血异常。

Hemostatic abnormalities associated with monoclonal gammopathies.

作者信息

Robert F, Mignucci M, McCurdy S A, Maldonado N, Lee J Y

机构信息

Medical Service, Hematology-Oncology Section, VA Medical Center, San Juan, Puerto Rico.

出版信息

Am J Med Sci. 1993 Dec;306(6):359-66. doi: 10.1097/00000441-199312000-00001.

Abstract

To confirm and expand previous observations about the association of monoclonal gammopathies with hemostatic defects, a prospective evaluation was made in 42 patients with lymphoplasmacytic disorders. The incidence of bleeding complications was low, despite the diversity of abnormal hemostatic tests observed in these patients. Patients with myeloma frequently had abnormal coagulation tests, including thrombin time (64%), fibrin degradation products (32%), platelet aggregation tests with different agonist (30% to 55%), and bleeding time (22%). The lack of platelet response to ristocetin and normal ristocetin cofactor activity in four patients with myeloma may suggest a Bernard-Soulier-type defect. Serum viscosity was negatively correlated with platelet aggregation with collagen, ristocetin, and adenosine diphosphate. In patients with immunoglobulin myeloma, there was a positive correlation between an increased viscosity and a prolonged thrombin time. This study demonstrates the wide variety of coagulation abnormalities in lymphoplasmacytic disorders, usually without significant clinical implications.

摘要

为了证实并扩展先前关于单克隆丙种球蛋白病与止血缺陷相关性的观察结果,对42例淋巴浆细胞疾病患者进行了前瞻性评估。尽管在这些患者中观察到多种异常止血试验,但出血并发症的发生率较低。骨髓瘤患者经常出现异常凝血试验,包括凝血酶时间(64%)、纤维蛋白降解产物(32%)、不同激动剂的血小板聚集试验(30%至55%)以及出血时间(22%)。4例骨髓瘤患者对瑞斯托霉素缺乏血小板反应且瑞斯托霉素辅因子活性正常,这可能提示存在伯纳德-苏利耶型缺陷。血清黏度与胶原、瑞斯托霉素和二磷酸腺苷诱导的血小板聚集呈负相关。在免疫球蛋白骨髓瘤患者中,黏度增加与凝血酶时间延长呈正相关。本研究表明淋巴浆细胞疾病存在多种凝血异常,通常无显著临床意义。

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