Siaka C, Rugeri L, Caron C, Goudemand J
Laboratoire d'Hématologie, Hôpital Cardiologique, Centre Hospitalier Regional Universitaire of Lille, France.
Haemophilia. 2003 May;9(3):303-8. doi: 10.1046/j.1365-2516.2003.00750.x.
The pathophysiology of acquired von Willebrand syndrome (AVWS), a rare bleeding disorder, is not fully understood. Circulating antibodies to Von Willebrand factor (VWF) are found in patients with AVWS associated with lymphoproliferative disorders but these autoantibodies are difficult to detect with routine laboratory tests and neutralisation assays. We have developed a simple enzyme-linked immunosorbent assay (ELISA) to detect serum antibody binding to VWF protein immobilized on polystyrene plates. Ten patients with AVWS were studied, eight of whom also had lymphoproliferative disorders. We found antibodies in eight patients; all of them were positive for IgG and five were also positive for IgM. This simple method appears to be more sensitive than functional assays, which failed to identify two of the patients who were positive with the ELISA. In conjunction with other tests, this ELISA method may be useful for demonstrating the immunological mechanism underlying some cases of AVWS. Such patients would qualify for intravenous immunoglobulin therapy, which can correct the clotting disorder.
获得性血管性血友病综合征(AVWS)是一种罕见的出血性疾病,其病理生理学尚未完全明确。在与淋巴增生性疾病相关的AVWS患者中可发现针对血管性血友病因子(VWF)的循环抗体,但这些自身抗体难以通过常规实验室检测和中和试验检测到。我们开发了一种简单的酶联免疫吸附测定(ELISA)方法,用于检测血清抗体与固定在聚苯乙烯板上的VWF蛋白的结合。对10例AVWS患者进行了研究,其中8例还患有淋巴增生性疾病。我们在8例患者中发现了抗体;所有患者的IgG均呈阳性,5例患者的IgM也呈阳性。这种简单的方法似乎比功能测定更敏感,功能测定未能识别出ELISA检测呈阳性的2例患者。结合其他检测,这种ELISA方法可能有助于阐明某些AVWS病例的免疫机制。此类患者有资格接受静脉注射免疫球蛋白治疗,该治疗可纠正凝血障碍。