Open AccessTwo Cases of Veno-occlusive Disease/Sinusoidal Obstruction Syndrome After Thioguanine Treatment for Acute Lymphoblastic Leukemia
Author(s) -
Bernd Gruhn,
Grit Brodt,
HansJoachim Mentzel,
Jochen Ernst
Publication year - 2021
Publication title -
journal of pediatric hematology/oncology
Language(s) - English
Resource type - Journals
eISSN - 1536-3678
pISSN - 1077-4114
DOI - 10.1097/mph.0000000000002172
Subject(s) - defibrotide , medicine , hepatic veno occlusive disease , chemotherapy , complication , hematopoietic stem cell transplantation , gastroenterology , acute leukemia , leukemia , ascites , surgery , disease
Veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is a potentially life-threatening complication of hematopoietic cell transplantation conditioning or high-dose chemotherapy. The underlying pathogenesis involves toxic injury to hepatocytes and sinusoidal endothelial cells. Presenting symptoms include ascites, weight gain, hepatomegaly, and hyperbilirubinemia. Severe VOD/SOS with multiorgan failure has a mortality rate of >80% if left untreated. Thioguanine, a chemotherapy drug used to treat acute lymphoblastic leukemia, has been shown to cause VOD/SOS. Here, we describe cases of 2 patients who developed very severe VOD/SOS after starting thioguanine for acute lymphoblastic leukemia; both achieved complete remission with defibrotide and experienced no defibrotide-related adverse events.