Veno‐occlusive disease in pediatric patients affected by Wilms tumor

Abstract

PAI-1 levels were abnormal in WT-VOD. Defibrotide was a safe, well-tolerated, and potentially efficacious therapy in this group of patients. Further prospective study is needed in WT-VOD to confirm these data.

Sinusoidal Obstruction Syndrome (Hepatic Veno-Occlusive Disease)

“…118 For pediatric patients developing SOS after chemotherapy and hepatectomy, careful supportive measures and anti-fibrinolytic drugs are the standard treatment, 74,75 In one report, two patients with life-threatening SOS who failed to respond to the standard therapeutic approach were successfully treated with non-activated protein C supplementation. 124 …”

“…In the pediatric population, chemotherapy accompanying hepatectomy for metastatic solid cancer such as Wilms tumor may also cause hepatic SOS. 74,75 Oxaliplatin is a key agent in such regimes, and may cause sinusoidal injury, onset of SOS, and increased morbidity before or after hepatectomy. [76][77][78] Histologic evidence of high-grade lesions of SOS has been reported to be present in 59% of patients receiving preoperative oxaliplatin treatment before hepatectomy, based on examination of the surrounding resected liver.…”

Preoperative Chemotherapy With Doxorubicin in Wilms Tumor

“…8 Actinomycin-D is one of the drugs considered responsible for the development of hepatic venooclusive disease. 9 In a series of 206 patients with WT, the incidence of venooclusive disease was reported to be 5%. 10 Actinomycin-D toxicity rates were found to be higher, especially in children younger than 12 months.…”

Dactinomycin-induced Hepatic Sinusoidal Obstruction Syndrome Responding to Treatment with N-acetylcysteine

“…Pharmacologic intervention for chemotherapy-induced hepatic SOS has previously been reported in nine cases (Table 1) 19-23. The therapeutic agents include anticoagulants such as defibrotide, antithrombin III, heparin, protein C concentrate, and gabexate mesylate alone or in various combinations.…”