Veno-occlusive disease with multi-organ involvement following actinomycin-D

Actinomycin-D (Act-D) is a rare cause of veno-occlusive disease (VOD). Between 1993 and 1998, we managed 6 patients, all male, median age 19 months (range 6-48 months) who received Act-D for Wilms' tumour (n = 4), clear cell sarcoma (n = 1) or rhabdomyosarcoma (n = 1). VOD presented with a median platelet count of 12×109/l, INR 3.8, fibrinogen 16 mg/l, fibrinogen degradation products (FDPs) ≥80 μg/l, aspartate aminotransferase (AST) 6922 IU/l, bilirubin 47 μmol/l. In 3 cases, transient liver dysfunction and thrombocytopenia without neutropenia had been observed after a previous course of Act-D. All six children developed encephalopathy, hepatomegaly, ascites, reversed portal flow and renal impairment. All received mechanical ventilation and two required haemofiltration. The treatment was supportive. Severe Adult Respiratory Distress Syndrome developed in 3 patients, all of whom died. 3 patients recovered. The outcome of VOD with multi-organ failure is poor. Intravascular coagulopathy precedes and characterises severe VOD during Act-D treatment.