Pihusch M, Wegner H, Goehring P, Salat C, Pihusch V, Andreesen R, Kolb H-J, Holler E, Pihusch R
Abteilung fuer Haematologie und Internistische Onkologie, Klinikum der Universitaet Regensburg, Regensburg, Germany.
Bone Marrow Transplant. 2005 Oct;36(7):631-7. doi: 10.1038/sj.bmt.1705114.
Veno-occlusive disease (VOD) is one of the most serious complications following hematopoietic stem cell transplantation (HSCT) and is associated with a high mortality. We conducted a large trial on the clinical significance of protein C (PC) and procollagen III peptide (PNPIII) levels, which have been described as possible diagnostic markers of VOD. In total, 350 patients undergoing allogeneic HSCT were included. PC and PNPIII levels were analyzed prior to conditioning and weekly until 8 weeks after the HSCT. Signs of VOD and other transplantation-related complications (graft-versus-host disease (GVHD), toxicity, microangiopathic hemolytic anemia, infection) were recorded weekly throughout the trial. Patients showed a significant drop of the PC levels in VOD (70.3 vs 96.3%, P<0.001) and with increasing severity of aGVHD. Steroids increased the PC levels (69.4% vs 109.4%, P<0.001). The highest PNPIII levels were registered in patients with VOD (mean 6.3 IU/ml). Patients with aGVHD showed an elevation of PNPIII, especially patients with hepatic aGVHD. PC levels during conditioning do not predict VOD (98.5 vs 76.5%, NS). Although PC and PNPIII may play a role in the pathogenesis of VOD they cannot discriminate between complications with jaundice and are only of limited help in the differential diagnosis of VOD.
静脉闭塞性疾病（VOD）是造血干细胞移植（HSCT）后最严重的并发症之一，且死亡率很高。我们针对蛋白C（PC）和Ⅲ型前胶原肽（PNPIII）水平的临床意义进行了一项大型试验，这两种物质已被描述为VOD可能的诊断标志物。总共纳入了350例接受异基因HSCT的患者。在预处理前以及直至HSCT后8周每周分析PC和PNPIII水平。在整个试验过程中每周记录VOD的体征以及其他与移植相关的并发症（移植物抗宿主病（GVHD）、毒性、微血管病性溶血性贫血、感染）。VOD患者的PC水平显著下降（70.3%对96.3%，P<0.001），且随着急性GVHD严重程度的增加而下降。类固醇使PC水平升高（69.4%对109.4%，P<0.001）。VOD患者的PNPIII水平最高（平均6.3 IU/ml）。急性GVHD患者的PNPIII升高，尤其是肝脏急性GVHD患者。预处理期间的PC水平不能预测VOD（98.5%对76.5%，无显著性差异）。尽管PC和PNPIII可能在VOD的发病机制中起作用，但它们无法区分伴有黄疸的并发症，在VOD的鉴别诊断中帮助有限。
