Authors:
Kotecha RS, Buckland A, Phillips MB, Cole CH, Gottardo NG
Authors notes:
Journal of Pediatric Hematology/Oncology 36(1): 76-80.
Keywords:
childhood, hepatic sinusoidal obstruction syndrome, medulloblastoma, veno-occlusive disease
Abstract:
Hepatic sinusoidal obstruction syndrome (HSOS), also known as veno-occlusive disease, is a well-recognized toxic complication after autologous and allogeneic hematopoietic stem cell transplant, during treatment of Wilms tumor and rhabdomyosarcoma associated with actinomycin-D, and during acute lymphoblastic leukemia therapy due to oral 6-thioguanine.
However, its occurrence in the context of chemotherapy regimens for other childhood malignancies is rare.
We report a 5-year-old girl with high-risk anaplastic medulloblastoma, who developed severe HSOS during her second cycle of maintenance chemotherapy, consisting of vincristine, cisplatin, and cyclophosphamide.
She was treated with defibrotide with complete resolution of the HSOS.
These findings and a review of the literature, highlight the occurrence of HSOS in children outside the established settings of hematopoietic stem cell transplantation, Wilms tumor, rhabdomyosarcoma, and acute lymphoblastic leukemia.