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

作者：Farruggia Piero; Macaluso Alessandra; Tropia Serena; Di Marco Floriana; Russo Delia; Grigoli Antonella; Trizzino Antonino; D'Angelo Paolo^*

来源：Pediatric Hematology and Oncology, 2011, 28(3): 237-243.

DOI：10.3109/08880018.2010.535118

• actinomycin D
• hepatopathy-thrombocytopenia syndrome
• vein occlusive disease
• Wilms Tumor

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 xx 10<SU3</SU/mu mu L). 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.</.

• 出版日期2011-4