Clinical Outcomes of Hepatic Veno-Occlusive Disease( VOD)/Sinusoidal Obstruction Syndrome(SOS) after Hematopoietic Stem Cell Transplant(HSCT): Single Center Study = Clinical Outcomes of Hepatic Veno-Occlusive Disease( VOD)/Sinusoidal Obstruction Syndrome(SOS) after Hematopoietic Stem Cell Transplant(HSCT): Single Center Study

다국어 초록 (Multilingual Abstract)

Aims: Hepatic veno-occlusive disease(VOD), also called sinusoidal obstruction syndrome(SOS), is a potentially life threatening complication primarily associated with hematopoietic cell transplantation( HSCT). VOD/SOS presents with hyperbilirubinemia, ascites, weight gain and painful hepatosplenomegaly. VOD/ SOS with multiorgan failure(MOF) is associated with a high mortality rate (>80%). Defibrotide(25mg/kg/day) is approved to treat hepatic VOD/SOS with renal or pulmonary dysfunction post HSCT in the United States.
Methods: Patients who were diagnosed with VOD post HSCT between 2007~2018 were retrospectively reviewed. Patients were diagnosed by Baltimore or modified Seattle criteria or biopsy. Patients received defibrotide or supportive treatment for treatment of VOD.
Results: The total number of transplants between 2007~2018 was 800 and 30 patients were diagnosed with VOD. The incidence of VOD was 4%. 7(30.4%) patients were diagnosed within 21 days post HSCT(early onset VOD), and 23(76.7%) patients were diagnosed after 21 days post HSCT(late onset VOD). Post HSCT 100 day survival rates were 70% among VOD patients(n=30), 85.7% among early onset VOD patients(n=7), and 65.2% among late onset VOD patients(n=23). 8(26.7%) patients developed severe VOD and it occurred late onset after 21days post HSCT. 7 patients among severe VOD patients(n=8) received Defibrotide treatment. Survival at day +100 post-HSCT were 75% for patients with severe VOD and 83.4% for severe VOD patients treated with Defibrotide. Survival at day +100 post-HSCT for VOD patients treated with defibrotide(n=13) and patients treated with supportive care(n=17) were 53.8% and 82.4% respectively. 4(30.7%) patients out of 13 patients who received Defibrotide treatment developed bleeding complications.
Conclusions: The incidence of VOD was 4% and severe VOD developed at late onset(> Post HSCT 21 days). 26.7% of VOD patients developed severe VOD. Defibrotide treatment did not increase day +100 post-HSCT survival rates.