Abstract:
The current review provides an overview of the thrombotic risk observed in patients with MG who do not otherwise require treatment. We discuss clinical and biomarker studies that highlight the heterogenous hemostatic profile observed in these patients and how knowledge has evolved over the past 20 years. Biomarker studies suggest shared biologic features between multiple myeloma and monoclonal gammopathy of undetermined significance (MGUS), which involves both hypercoagulability and platelet activation. Hemostatic abnormalities identified in MGUS patients cannot be translated into clinical practice as they lack correlation to clinical events. The prothrombotic phenotype of MGUS patients has not been ascertained yet, but novel data on coagulation markers are promising. We also review rare conditions associated with the thrombogenic properties of the monoclonal protein that predispose to arterial, venous or microthrombotic events and demonstrate that the M-protein can be linked to clinically significant thrombotic events. Cryoglobulinemia, cryofibrinogenemia, cryo-crystaloglobulinemia and MG-related antiphospholipid syndrome are reviewed. We propose the new umbrella term \"monoclonal gammopathy of thrombotic significance\" (MGTS) to refer to significant, recurrent thrombotic events in patients with MGUS that provide a rationale for targeting the underlying plasma cell clone. Identifying MGUS patients at high risk for thrombotic events is currently a challenge.
摘要:
本综述概述了在不需要其他治疗的MG患者中观察到的血栓形成风险。我们讨论了临床和生物标志物研究,这些研究强调了在这些患者中观察到的异质止血特征以及过去20年知识的演变。生物标志物研究表明,多发性骨髓瘤和意义不明的单克隆丙种球蛋白病(MGUS)之间具有共同的生物学特征。这涉及高凝和血小板活化。MGUS患者中发现的止血异常不能转化为临床实践,因为它们与临床事件缺乏相关性。MGUS患者的血栓前表型尚未确定,但是关于凝血标志物的新数据是有希望的。我们还回顾了与易患动脉的单克隆蛋白的血栓形成特性相关的罕见疾病,静脉或微血栓事件,并证明M蛋白可以与临床上重要的血栓事件相关联。冷球蛋白血症,冷冻纤维蛋白原血症,综述了冷冻结晶球蛋白血症和MG相关的抗磷脂综合征。我们提出了新的总称“具有血栓性意义的单克隆丙种球蛋白病”(MGTS)来指代重要的,MGUS患者的复发性血栓事件为靶向潜在的浆细胞克隆提供了理论基础.目前,确定处于血栓形成事件高风险的MGUS患者是一个挑战。