血清 vWF 抗原水平升高与预后不良相关，而循环 ADAMTS-13 抗原水平降低与 IgM 水平升高和 WM 特征相关，但与症状性 WM 患者的 vWF 水平升高无关。
Elevated vWF Antigen Serum Levels Are Associated With Poor Prognosis, and Decreased Circulating ADAMTS-13 Antigen Levels Are Associated With Increased IgM Levels and Features of WM but not Increased vWF Levels in Patients With Symptomatic WM.
机构信息
Department of Oncology, Alexandra Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece; Department of Clinical Therapeutics, Alexandra Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece.
Department of Oncology, Alexandra Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece; Department of Clinical Therapeutics, Alexandra Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece.
出版信息
Clin Lymphoma Myeloma Leuk. 2019 Jan;19(1):23-28. doi: 10.1016/j.clml.2018.07.294. Epub 2018 Aug 23.
BACKGROUND
Waldenström's macroglobulinemia (WM) is a rare malignancy characterized by bone marrow infiltration by lymphoplasmacytic cells and the presence of a monoclonal IgM paraprotein. The interactions of lymphoplasmacytic cells with other cells in their microenvironment, including mast cells and endothelial cells, support their survival and proliferation and can induce resistance to therapy. von Willebrand factor (vWF) plays a key role in primary hemostasis but is also a marker of endothelial "stimulation." High levels of vWF have been associated with an adverse prognosis in patients with symptomatic WM and might reflect the interactions between lymphoplasmacytic cells and other cells of their microenvironment.
MATERIALS AND METHODS
Considering vWF and ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) as markers of endothelial dysfunction and activation, we evaluated the prognostic importance of vWF and ADAMTS-13 antigen levels in the serum of patients with previously untreated symptomatic WM to validate vWF as a possible prognostic marker for progression-free and overall survival. We also validated the measurement of vWF in the serum instead of citrated plasma and investigated the possible correlations of ADAMTS-13 antigen levels with disease characteristics. The analysis included 42 patients with symptomatic WM and 19 matched healthy controls.
RESULTS
The serum levels of vWF antigen provided significant prognostic information, and patients with levels ≥ 200 IU/dL had a very poor prognosis compared with patients with lower levels. The ADAMTS-13 antigen levels were decreased in WM patients and correlated with the IgM levels, β-microglobulin, and extent of bone marrow infiltration.
CONCLUSION
vWF levels measured in the serum could become an important prognostic marker in patients with WM and requires further investigation.
背景
华氏巨球蛋白血症（WM）是一种罕见的恶性肿瘤，其特征为骨髓中淋巴浆细胞浸润和单克隆 IgM 副蛋白的存在。淋巴浆细胞与其微环境中的其他细胞（包括肥大细胞和内皮细胞）相互作用，支持其存活和增殖，并可诱导对治疗的耐药性。血管性血友病因子（vWF）在初次止血中起关键作用，但也是内皮“刺激”的标志物。vWF 水平升高与有症状 WM 患者的不良预后相关，并且可能反映了淋巴浆细胞与其微环境中其他细胞的相互作用。
材料和方法
鉴于 vWF 和 ADAMTS-13（一种具有血小板反应蛋白 1 型基序的解整合素金属蛋白酶 13）作为内皮功能障碍和激活的标志物，我们评估了未经治疗的有症状 WM 患者血清中 vWF 和 ADAMTS-13 抗原水平对无进展生存期和总生存期的预后重要性，以验证 vWF 作为无进展生存期和总生存期的可能预后标志物。我们还验证了在血清中测量 vWF 而不是在枸橼酸盐血浆中测量 vWF，并研究了 ADAMTS-13 抗原水平与疾病特征的可能相关性。该分析包括 42 例有症状 WM 患者和 19 例匹配的健康对照者。
结果
vWF 抗原的血清水平提供了重要的预后信息，并且水平≥200 IU/dL 的患者与水平较低的患者相比预后非常差。WM 患者的 ADAMTS-13 抗原水平降低，并且与 IgM 水平、β-微球蛋白和骨髓浸润程度相关。
结论
在 WM 患者中，血清中测量的 vWF 水平可能成为重要的预后标志物，需要进一步研究。
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