Veno-occlusive disease of the liver after combined adjuvant chemotherapy for a 1-year-old boy with rhabdomyosarcoma: potential usefulness of the gabexate mesylate (FOY).

A tumor in the left thigh of a 1-year-old boy was diagnosed as rhabdomyosarcoma (stage 1). The tumor was excised and vincristine (1.5 mg/m2 given twice), actinomycin D (15 micrograms/kg given five times), and cyclophosphamide (600 mg/m2 given once) were prescribed. On the 12th day after initiating this chemotherapy, his liver rapidly enlarged and ascites, pleural effusion, and disturbance of consciousness due to hepatic failure successively appeared. Before these clinical signs appeared, laboratory data demonstrated enhanced coagulation and fibrinolysis such as thrombocytopenia refractory to platelet transfusion and a marked increase in fibrinogen and fibrin degradation product (FDP). Hepatic veno-occlusive disease (VOD) was suspected, and based on the laboratory data indicating enhanced coagulation, anticoagulant (gabexate mesylate; FOY), diuretic, and supportive therapy for hepatic failure was begun. The clinical course was favorable and recovery was complete in 2 months, with no sequelae. We propose that platelet count, coagulation factor levels, and FDP may be useful for the early detection of VOD, and an anticoagulant drug such as FOY is worthy of consideration for the prevention and treatment of VOD.