Salat C, Holler E, Schleuning M, Eisele B, Reinhardt B, Kolb H, Pihusch R, Domrath R, Hiller E
Department of Hematology and Oncology, Medical Clinic III, Klinikum Grosshadern, Munich, Germany.
Ann Hematol. 1995 Dec;71(6):271-4. doi: 10.1007/BF01697978.
Capillary leak syndrome (CLS) is a severe complication after bone marrow transplantation (BMT). To investigate whether there is a pathogenetic role of the complement system, we monitored the levels of the terminal complement complex C5b-9 (TCC) and C3a-desArg as indicators of an activation of the complement system and the inhibitor of the classical pathway of the complement cascade, C1 inhibitor (C1-INH), in 48 bone marrow transplant recipients from 1 week before to 5 weeks after transplantation. Capillary leak syndrome developed in 7 out of 48 patients between days 1 and 12 after BMT. Complement activation as indicated by TCC levels was more pronounced in patients with CLS (n = 7) from day -8 to +28 (p < 0.05; day -1) and the elevation of TCC levels lasted longer in CLS patients (peak day 21) than in patients without this complication (peak day 7). Mean C3a-desArg levels were highest in patients with CLS reaching a peak at day 7. During the early posttransplant period a significant elevation of C1-INH levels (p < 0.01 and p < 0.05 respectively) compared with baseline levels (day -8) was found in patients with and without CLS, which was more pronounced in those patients with CLS (p < 0.05). Although we could not observe an absolute C1-INH deficiency as compared to healthy individuals our data support the presence of a relative deficiency of the inhibitor which might explain the reported beneficial effects of C1-INH substitution in BMT related CLS.
毛细血管渗漏综合征（CLS）是骨髓移植（BMT）后的一种严重并发症。为了研究补体系统是否具有致病作用，我们监测了48例骨髓移植受者从移植前1周到移植后5周期间终末补体复合物C5b - 9（TCC）和C3a - desArg的水平，作为补体系统激活的指标，以及补体级联反应经典途径的抑制剂C1抑制剂（C1 - INH）的水平。48例患者中有7例在BMT后第1天至第12天发生了毛细血管渗漏综合征。TCC水平所表明的补体激活在CLS患者（n = 7）中从第 - 8天至 + 28天更为明显（p < 0.05；第 - 1天），并且CLS患者中TCC水平的升高持续时间更长（峰值在第21天），比没有这种并发症的患者（峰值在第7天）持续时间长。CLS患者的平均C3a - desArg水平最高，在第7天达到峰值。在移植后的早期，有CLS和无CLS的患者与基线水平（第 - 8天）相比，C1 - INH水平均显著升高（分别为p < 0.01和p < 0.05），在有CLS的患者中更为明显（p < 0.05）。尽管与健康个体相比，我们未观察到绝对的C1 - INH缺乏，但我们的数据支持存在抑制剂的相对缺乏，这可能解释了报道的C1 - INH替代在BMT相关CLS中的有益作用。
