Hepatopathy-thrombocytopenia Syndrome (HTS) after Actinomycin-D Therapy: Report of Three Cases and Review of the Literature

• Published in: Pediatric hematology and oncology Vol. 28; no. 3; pp. 237 - 243
• Main Authors: Farruggia, Piero, Macaluso, Alessandra, Tropia, Serena, Di Marco, Floriana, Russo, Delia, Grigoli, Antonella, Trizzino, Antonino, D'Angelo, Paolo
• Format: Journal Article
• Language: English
• Published: England Informa Healthcare 01.04.2011; Taylor & Francis
• Subjects: actinomycin D; Antibiotics, Antineoplastic - adverse effects; Chemical and Drug Induced Liver Injury; Dactinomycin - adverse effects; Female; hepatopathy-thrombocytopenia syndrome; Humans; Infant; Kidney Neoplasms - drug therapy; Male; Review Literature as Topic; Syndrome; Thrombocytopenia - chemically induced; vein occlusive disease; Wilms Tumor; Wilms Tumor - drug therapy
• ISSN: 0888-0018; 1521-0669
• DOI: 10.3109/08880018.2010.535118

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 × 103/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.