Treatment of Sinusoidal Obstruction Syndrome With Defibrotide in Pediatric Cancer Patients Following Nontransplant-associated Chemotherapy: A Case Report and Review of the Literature

Abstract

Sinusoidal obstruction syndrome (SOS), formerly venoocclusive disease (VOD), in pediatric cancer patients often presents as a complication of hematopoietic stem cell transplantation, and less commonly secondary to nontransplant-associated chemotherapy. Therapy with defibrotide is well-described as standard care for transplant-associated SOS/VOD, but the treatment of nontransplant-associated SOS/VOD is less clear. We report a 3-year-old with relapsed Wilms tumor and recurrent SOS/VOD, with successful use of defibrotide during chemotherapy. A review of pediatric cancer patients with nontransplant-associated SOS/VOD treated with defibrotide revealed 83 patients, and 66 were in remission. This review supports early treatment with defibrotide in patients with nontransplant-associated SOS/VOD.

Sinusoidal Obstruction Syndrome of the Liver Associated With 6-Mercaptopurine During Maintenance in a Child With T-cell Acute Lymphoblastic Leukemia

“…In addition, these reports generally describe events as occurring either with a definitive comorbidity (ie, BK virus and the use of ciprofloxacin treatment) or in the context of more extensive chemotherapy (ie, intensive concomitant asparaginase therapy), are mild-moderate in severity, and self-resolve after discontinuation of 6-MP 5,6. Reports describing non-HSCT SOS severe enough to require defibrotide do not include 6-MP as a risk factor 2,17. The exact etiology of our patient’s SOS was unclear, but we assume the driving cause was his 6-MP exposure as he had few other identified potential causes.…”

“…5,6 Reports describing non-HSCT SOS severe enough to require defibrotide do not include 6-MP as a risk factor. 2,17 The exact etiology of our patient's SOS was unclear, but we assume the driving cause was his 6-MP exposure as he had few other identified potential causes. Genotype testing for TPMT and NUDT15 was normal, although this does not fully rule out other less common polymorphisms that may affect 6-MP metabolism.…”