Hodak E, Trattner A, David M, Kornbrot N, Modan B, Lurie H, Lawrie A, Harrison P, Sandbank M, Inbal A
Department of Dermatology, Beilinson Medical Center, Petah Tiqva, Israel.
J Am Acad Dermatol. 1993 Feb;28(2 Pt 1):217-21. doi: 10.1016/0190-9622(93)70030-w.
von Willebrand factor (vWF) is synthesized almost exclusively by endothelial cells and is stored there as ultra-high-molecular-weight multimers. The vWF multimers that are detected in the plasma are smaller than those stored within the endothelium. In two previous studies, comprising small series of cases with classic Kaposi's sarcoma (KS), an endothelium-derived tumor, increased levels of plasma von Willebrand factor antigen (VWF:Ag, the antigenic structure) were reported, suggesting that vWF:Ag may be a marker of endothelium proliferation.
Our purpose was to investigate the quantitative as well as qualitative alterations of plasma vWF in a large series of patients with classic KS at various stages of the disease.
Levels of plasma vWF:Ag were studied in 38 patients with classic KS confined to the skin at various stages of the disease and compared with a control group. Thirty-three patients had active KS (i.e., with skin lesions) and five were in remission. In five patients with active KS multimeric analysis of plasma vWF was also performed.
The levels of vWF:Ag were significantly higher among KS patients than in the control group (n = 29, p < 0.01). Levels of vWF:Ag were also significantly higher in patients with active disease as compared with those in remission (p < 0.05). No correlation was found between vWF:Ag levels and the extent of KS. Analysis of the multimeric pattern of plasma vWF showed enhanced staining of all bands, particularly the intermediate and high molecular weight forms, which resemble the endothelial forms as opposed to normal circulating vWF multimers.
Quantitative as well as qualitative alterations in plasma vWF were found in patients with KS, which may reflect the destruction or activation of endothelial cells within the lesions. vWF:Ag may serve as a marker of disease activity in classic KS; however, it is not a good marker for the extent of the disease.
血管性血友病因子（vWF）几乎完全由内皮细胞合成，并以超大分子量多聚体的形式储存于内皮细胞中。血浆中检测到的vWF多聚体比内皮细胞内储存的多聚体小。在之前两项针对少量经典卡波西肉瘤（KS，一种内皮源性肿瘤）病例的研究中，报告称血浆血管性血友病因子抗原（VWF:Ag，抗原结构）水平升高，提示VWF:Ag可能是内皮细胞增殖的标志物。
我们的目的是研究大量处于疾病不同阶段的经典KS患者血浆vWF的定量和定性改变。
研究了38例疾病不同阶段局限于皮肤的经典KS患者的血浆VWF:Ag水平，并与一个对照组进行比较。33例患者患有活动性KS（即有皮肤病变），5例处于缓解期。对5例活动性KS患者还进行了血浆vWF的多聚体分析。
KS患者的VWF:Ag水平显著高于对照组（n = 29，p < 0.01）。与缓解期患者相比，活动性疾病患者的VWF:Ag水平也显著更高（p < 0.05）。未发现VWF:Ag水平与KS范围之间存在相关性。血浆vWF多聚体模式分析显示所有条带的染色增强，尤其是中分子量和高分子量形式，与正常循环vWF多聚体相比，它们类似于内皮形式。
在KS患者中发现了血浆vWF的定量和定性改变，这可能反映了病变内内皮细胞的破坏或激活。VWF:Ag可能作为经典KS疾病活动的标志物；然而，它不是疾病范围的良好标志物。
