HEPATIC VENOOCCLUSIVE DISEASE WITH SEVERE CAPILLARY LEAKAGE AFTER PERIPHERAL STEM-CELL TRANSPLANTATION - TREATMENT WITH RECOMBINANT PLASMINOGEN-ACTIVATOR AND C1-ESTERASE INHIBITOR CONCENTRATE

Severe veno-occlusive disease (VOD), characterised by elevated serum b ilirubin levels, is a known complication in the first 3 weeks after pe ripheral blood stem cell transplantation (PBSCT). Severe VOD is associ ated with capillary leakage and multiple organ dysfunction and leads t o high mortality. We report a 17-year-old male, who developed VOD with capillary leakage (CL) after allogeneic PBSCT. The patient presented with a maximum serum bilirubin of 25.4 mg/dl, weight gain (10% of base line weight), generalized edema, cardiovascular insufficiency, complem ent activation, jaundice and a decreased AT and protein C functional a ctivity. After VOD and CL were diagnosed the patient was treated with recombinant human plasminogen activator (rt-PA) and C1 esterase-inhibi tor concentrate (C1-INH-C). The clinical symptoms resolved and the pat ient's status stabilized. The patient was in an adequate clinical stat e 5 months after transplantation. We noted that the combined therapy w ith rt-PA and C1-INH-C in this high-risk situation led to a resolution of VOD with CL.