Brugieres L, Hartmann O, Benhamou E, Zafrani E S, Caillaud J M, Patte C, Kalifa C, Flamant F, Lemerle J
Service de Pédiatrie, Institut Gustave-Roussy, Villejuif, France.
Bone Marrow Transplant. 1988 Jan;3(1):53-8.
Two hundred and thirty-six consecutive courses of high-dose chemotherapy with autologous bone marrow transplantation in children with solid tumors were reviewed in order to assess the incidence, clinical presentation and outcome of veno-occlusive disease (VOD) of the liver. Patients conditioned with total body irradiation were excluded from this study. Eleven patients (4.6%) met the diagnostic criteria for VOD. The clinical course included sudden weight gain, jaundice, hepatomegaly and ascites. Renal dysfunction and refractoriness to platelet transfusions occurred in the most severe forms. Seven patients recovered within 7-29 days of onset and four patients died, all with renal failure and fluid overload. The time of onset appeared to determine two patterns of outcome: mild forms with early onset (before day 11) and more severe forms with onset after day 17. Analysis of pretransplant factors revealed no significant association with an increased risk of VOD. However, all the patients with severe VOD had received a conditioning regimen containing cyclophosphamide which might be involved in the pathogenesis of VOD.
回顾了236例实体瘤患儿接受大剂量化疗及自体骨髓移植的连续疗程,以评估肝静脉闭塞病(VOD)的发生率、临床表现及转归。本研究排除了接受全身照射预处理的患者。11例患者(4.6%)符合VOD的诊断标准。临床过程包括体重突然增加、黄疸、肝肿大和腹水。最严重的形式出现肾功能不全和对血小板输注无效。7例患者在发病后7 - 29天内恢复,4例患者死亡,均死于肾衰竭和液体超负荷。发病时间似乎决定了两种转归模式:发病早(第11天之前)的轻症形式和第17天之后发病的重症形式。移植前因素分析显示与VOD风险增加无显著关联。然而,所有重症VOD患者均接受了含环磷酰胺的预处理方案,这可能与VOD的发病机制有关。