Abstract

PurposeHepatic sinusoidal obstruction syndrome (SOS) represents a serious complication following hematopoietic stem cell transplantation (HSCT). Our study aimed to investigate important risk factors of SOS in a pediatric population.MethodsThis retrospective study analyzed 105 children, adolescents and young adults who underwent allogeneic HSCT at our pediatric HSCT center in Jena. The observation period was 12 years and SOS was defined by the pediatric criteria of the European Society for Blood and Marrow Transplantation (EBMT).Results15 out of all 105 patients developed SOS (14.3%). The median time from HSCT to SOS diagnosis was 12 days. The mortality rate of SOS was 20.0%. In univariate analyses, we identified the significant risk factors of patient age < 1 year [odds ratio (OR) = 7.25, p = 0.037], prior treatment with gemtuzumab ozogamicin (OR = 11.00, p = 0.020), high pretransplant ferritin levels above 1500 ng/mL (OR = 4.00, p = 0.033), 2000 ng/mL (OR = 4.69, p = 0.016), and 2400 ng/mL (OR = 5.29, p = 0.005) as well as international normalized ratio (INR) ≥ 1.3 (OR = 5.91, p = 0.009). The following risk factors could be confirmed in multivariate analysis: treatment with gemtuzumab ozogamicin (OR = 9.24, p = 0.048), ferritin > 2400 ng/mL (OR = 5.74, p = 0.023), and INR ≥ 1.3 (OR = 8.02, p = 0.007).ConclusionOur study confirms several risk factors from the current literature. Additionally, this is the first report on the risk factor of high pretransplant INR.

Highlights

  • Hepatic sinusoidal obstruction syndrome (SOS), previously called veno-occlusive disease (VOD), is among the potentially life-threatening complications following hematopoietic stem cell transplantation (HSCT).Pathophysiologically, initial damage to the sinusoidal endothelium leads to an activation of endothelial cells (DeLeve et al 1999, 2002)

  • 5 out of 90 patients without the diagnosis of SOS died in this period, which results in 100-day mortality of only 5.6%

  • Pediatric European Society for Blood and Marrow Transplantation (EBMT) criteria were used corresponding to our study

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Summary

Introduction

Hepatic sinusoidal obstruction syndrome (SOS), previously called veno-occlusive disease (VOD), is among the potentially life-threatening complications following hematopoietic stem cell transplantation (HSCT). Initial damage to the sinusoidal endothelium leads to an activation of endothelial cells (DeLeve et al 1999, 2002). This damage is caused by factors like chemotherapy or radiotherapy as part of the conditioning regimen before HSCT. The frequency of SOS varies widely in the published literature depending on different diagnostic criteria (Carreras et al 2011; Coppell et al 2010; Kammersgaard et al 2019). New diagnostic criteria have been published on behalf of the European Society for Blood and Marrow Transplantation (EBMT) to achieve an

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