Low Incidence of hepatic sinusoidal obstruction syndrome/veno-occlusive disease in adults undergoing allogenic stem cell transplantation with prophylactic ursodiol and low-dose heparin

Lina Stutz,Jörg P Halter,Dominik Heim,Michael Medinger,Jakob R Passweg

doi:10.1038/s41409-021-01546-w

Lina Stutz, Jörg P Halter + Show 3 more

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https://doi.org/10.1038/s41409-021-01546-w

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Hepatic sinusoidal obstruction syndrome (SOS)/veno-occlusive disease (VOD) is a complication after allogenic hematopoietic stem-cell transplantation (allo-HSCT) with high mortality. The purpose of this study was to assess the incidence and outcome of SOS in patients after allo-HSCT with the impact of ursodeoxycholic acid (UDCA) and low-dose heparin as SOS prophylaxis. Out of 1016 patients, 23 developed SOS, with a cumulative incidence of 2.3% (95% CI 1.3–3.3) 6 months after HSCT. Approximately one quarter of these patients (26.1%) had late-onset SOS. A high proportion were very severe SOS cases (74%), and 83% of the patients were treated with defibrotide (DF). In multivariate analysis, advanced disease (p = 0.003), previous HSCT (p = 0.025) and graft versus host disease (GvHD) prophylaxis by post-transplant cyclophosphamide (PTCy) (p = 0.055) were associated with the development of SOS. The 1-year overall survival (OS) was significantly lower in the SOS group compared to patients without SOS (13% versus 70%, p = 0.0001). In conclusion, we found a low incidence of SOS in patients receiving low-dose heparin and UDCA prophylactically, but among SOS patients, a high mortality. Low-dose heparin and UDCA might be a prophylactic approach for SOS.

Highlights

Hepatic sinusoidal obstruction syndrome (SOS) (formerly venoocclusive disease (VOD)) is a potentially life-threatening complication after allogenic hematopoietic stem cell transplantation [1]
Between 2006 and 2020, 1,163 allo-HSCTs were performed at the University Hospital of Basel
Transfusion support and infection prophylaxis Red blood cell transfusion was given to patients with hemoglobin concentrations

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Hepatic sinusoidal obstruction syndrome (SOS) (formerly venoocclusive disease (VOD)) is a potentially life-threatening complication after allogenic hematopoietic stem cell transplantation (alloHSCT) [1]. An injury of the sinusoidal endothelium of the liver seems to be the underlying cause [1, 2], triggered by several factors including conditioning, drugs, allo-immunological reactions [3], and occurrence is favored by pre-existing liver damage [4]. Due to loss of integrity of the endothelium, erythrocytes, leukocytes and cellular debris penetrate into space of Disse [3], leading to an embolization of the centrilobular veins [2], resulting in hepatocellular damage and portal hypertension [5]. Clinical manifestations are unspecific [1], with hyperbilirubinemia, weight gain, painful hepatomegaly and ascites [5, 6]. The clinical course is ranging from mild forms spontaneously resolving, to severe forms with organ damage and multiorgan failure (MOF) [2]. The diagnosis is based on clinical presentation, supported by ultrasound, or, if the risk of invasive procedure is acceptable, confirmed histologically [1]

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