TY - JOUR
T1 - Veno-occlusive disease with multi-organ involvement following actinomycin-D
AU - Verdecchia, A.
AU - Mariotto, A.
AU - Capocaccia, R.
AU - Gatta, G.
AU - Micheli, A.
AU - Sant, M.
AU - Berrino, F.
PY - 2001
Y1 - 2001
N2 - 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.
AB - 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.
KW - Actinomycin-D
KW - Children
KW - Multi-organ failure
KW - Veno-occlusive disease
KW - Wilms' tumour
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UR - http://www.scopus.com/inward/citedby.url?scp=0035005246&partnerID=8YFLogxK
U2 - 10.1016/S0959-8049(01)00097-1
DO - 10.1016/S0959-8049(01)00097-1
M3 - Article
C2 - 11378345
AN - SCOPUS:0035005246
VL - 37
SP - 1141
EP - 1148
JO - European Journal of Cancer
JF - European Journal of Cancer
SN - 0959-8049
IS - 9
ER -