Glaspy J A
Bowyer Oncology Center, University of California, School of Medicine, Los Angeles.
Hematol Oncol Clin North Am. 1992 Dec;6(6):1301-14.
Patients with multiple myeloma, Waldenström's macroglobulinemia, benign monoclonal gammopathy, and other B-cell disorders associated with high titer serum paraproteins can manifest unique hemostatic disorders. Most of these disorders predispose the patient to hemorrhage, especially following surgical procedures. Mechanisms can include: acquired von Willebrand syndrome, paraprotein-induced platelet function defects, factor X deficiency, and local tissue fragility associated with amyloidosis, abnormalities of the function of fibrin, circulating anticoagulants, and thrombocytopenia. The mainstay of therapy is the treatment of the underlying disease. Depending on clinical circumstances, additional therapies might include: plasmapheresis with appropriate factor replacement, arginine vasopressin, fibrinolysis inhibitors, and splenectomy. Less commonly, the paraprotein disorders are associated with thrombotic complications, especially in those cases in which the lupus anticoagulant is present.
患有多发性骨髓瘤、华氏巨球蛋白血症、良性单克隆丙种球蛋白病以及其他与高滴度血清副蛋白相关的B细胞疾病的患者可能会出现独特的止血障碍。这些障碍大多使患者易发生出血，尤其是在外科手术后。其机制可能包括：获得性血管性血友病综合征、副蛋白诱导的血小板功能缺陷、因子X缺乏以及与淀粉样变性相关的局部组织脆性、纤维蛋白功能异常、循环抗凝物质和血小板减少。治疗的主要方法是治疗基础疾病。根据临床情况，其他治疗可能包括：进行血浆置换并补充适当的因子、精氨酸加压素、纤维蛋白溶解抑制剂和脾切除术。较少见的情况是，副蛋白疾病与血栓形成并发症相关，尤其是在存在狼疮抗凝物的病例中。
