Higashigawa M, Watanabe M, Nishihara H, Tabata N, Azuma E, Ido M, Ito M, Sakurai M
Department of Pediatrics, Mie University School of Medicine, Japan.
Leuk Res. 1995 Jul;19(7):477-80. doi: 10.1016/0145-2126(95)00009-d.
A 15-month-old boy with severe aplastic anemia developed veno-occlusive disease (VOD) after allogeneic bone marrow transplantation (BMT), in which the preparative regimen included 50 mg/kg/day cyclophosphamide and anti-lymphocyte globulin for 4 consecutive days. The diagnosis was made based on clinical symptoms and data including, hepatomegaly, right upper quadrant abdominal pain, jaundice, ascites, coagulopathy and thrombocytopenia which was refractory to transfusions of platelet concentrate. We gave 2, 3, 5 and 5 mg/day/body of recombinant tissue plasminogen activator (tPA) followed by heparin and prostaglandin E1 (PGE1) effectively and without significant side effect on days 9, 10, 13 and 14, respectively. Clinical and biochemical improvement was steady and dramatic. We suggest that tPA following continuous heparin and PGE1 infusion may be useful in the treatment of VOD even in infantile cases.
一名15个月大的患有严重再生障碍性贫血的男孩在异基因骨髓移植（BMT）后发生了静脉闭塞性疾病（VOD），其预处理方案包括连续4天给予50mg/kg/天的环磷酰胺和抗淋巴细胞球蛋白。诊断基于临床症状和数据，包括肝肿大、右上腹疼痛、黄疸、腹水、凝血障碍以及对浓缩血小板输注无效的血小板减少症。我们分别在第9、10、13和14天给予重组组织型纤溶酶原激活剂（tPA）2、3、5和5mg/天/体质量，随后给予肝素和前列腺素E1（PGE1），效果显著且无明显副作用。临床和生化指标持续且显著改善。我们认为，在持续输注肝素和PGE1后使用tPA可能对VOD的治疗有效，即使在婴儿病例中也是如此。
