title: Hepatopathy-thrombocytopenia syndrome (HTS) after actinomycin-D therapy: report of three cases and review of the literature.
abstract: Hepatopathy-thrombocytopenia syndrome (HTS) is a severe complication very similar to vein occlusive disease (VOD), also known as hepatic sinusoidal obstructive syndrome (SOS), characterized by fever, hepatopathy (hepatomegaly with abnormal liver function tests), ascites, weight gain, jaundice, and thrombocytopenia (platelet count less than 25 × 10(3)/μL). It has been generally observed in patients with Wilms tumor, and is commonly associated to administration of actinomycin D. We report three children with Wilms tumor, with severe HTS/SOS, but had a different outcome, in spite of vigorous supportive therapy.
authors.affiliation: Pediatric Hematology and Oncology, G. Di Cristina Children's Hospital, Italy.
authors.forename: Piero
authors.initials: P
authors.lastname: Farruggia
authors.forename: Alessandra
authors.initials: A
authors.lastname: Macaluso
authors.forename: Serena
authors.initials: S
authors.lastname: Tropia
authors.forename: Floriana
authors.initials: F
authors.lastname: Di Marco
authors.forename: Delia
authors.initials: D
authors.lastname: Russo
authors.forename: Antonella
authors.initials: A
authors.lastname: Grigoli
authors.forename: Antonino
authors.initials: A
authors.lastname: Trizzino
authors.forename: Paolo
authors.initials: P
authors.lastname: D'Angelo
mesh_terms: D000903:Antibiotics, Antineoplastic / Q000009:adverse effects; D056486:Chemical and Drug Induced Liver Injury; D003609:Dactinomycin / Q000009:adverse effects*; D005260:Female; D006801:Humans; D007223:Infant; D007680:Kidney Neoplasms / Q000188:drug therapy*; D008297:Male; D012196:Review Literature as Topic; D013577:Syndrome; D013921:Thrombocytopenia / Q000139:chemically induced*; D009396:Wilms Tumor / Q000188:drug therapy*
chemical_list: D000903:Antibiotics, Antineoplastic; D003609:Dactinomycin