Two Cases of Veno-occlusive Disease/Sinusoidal Obstruction Syndrome After Thioguanine Treatment for Acute Lymphoblastic Leukemia

J Pediatr Hematol Oncol. 2022 Mar 1;44(2):e572-e575. doi: 10.1097/MPH.0000000000002172.

Abstract

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.

MeSH terms

  • Endothelial Cells
  • Hematopoietic Stem Cell Transplantation* / adverse effects
  • Hepatic Veno-Occlusive Disease* / etiology
  • Humans
  • Polydeoxyribonucleotides / therapeutic use
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma* / complications
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma* / drug therapy
  • Thioguanine / adverse effects

Substances

  • Polydeoxyribonucleotides
  • Thioguanine