Diagnosis and severity criteria for sinusoidal obstruction syndrome/veno-occlusive disease in adult patients: a refined classification from the European society for blood and marrow transplantation (EBMT)

Abstract

Sinusoidal obstruction syndrome, also known as veno-occlusive disease (SOS/VOD), is a potentially life-threatening complication that can develop after hematopoietic cell transplantation (HCT). A new definition for diagnosis, and a severity grading system for SOS/VOD in adult patients, was reported a few years ago on behalf of the European Society for Blood and Marrow Transplantation (EBMT). The aim of this work is to update knowledge regarding diagnosis and severity assessment of SOS/VOD in adult patients, and also its pathophysiology and treatment. In particular, we now propose to refine the previous classification and distinguish probable, clinical and proven SOS/VOD at diagnosis. We also provide an accurate definition of multiorgan dysfunction (MOD) for SOS/VOD severity grading based on Sequential Organ Failure Assessment (SOFA) score.

Impact of inotuzumab ozogamicin on outcome in relapsed or refractory acute B-cell lymphoblastic leukemia patients prior to allogeneic hematopoietic stem cell transplantation and risk of sinusoidal obstruction syndrome/venous occlusive disease

“…In addition, risk of SOS/VOD is not negligible, necessitating continuous monitoring, recognition of SOS/VOD risk factors, such as double alkylating agents as conditioning regimen prior to allo-HCT and guidelines application to mitigate its incidence. 38 In particular, the addition of a second alkylating agent into the conditioning regimen must be avoided.…”

Prevention and management of acute toxicities from conditioning regimens during hematopoietic stem cell transplantation

“…Increases in LSM at Day +14 allowed for early detection of VOD/SOS with specificity of 99% and, as such, this can be a useful tool in VOD/SOS diagnosis, and may eventually be incorporated into future diagnostic criteria. 76 While VOD/SOS symptoms have been well described in the existing literature, none are specific to VOD/SOS, which often makes diagnosis challenging, particularly since it is based on retrospectively developed clinical criteria. The two validated diagnostic criteria for VOD/SOS are the Baltimore and the modified Seattle criteria, which differ in the number and magnitude of clinical criteria that are required for diagnosis.…”

“…Increases in LSM at Day +14 allowed for early detection of VOD/SOS with specificity of 99% and, as such, this can be a useful tool in VOD/SOS diagnosis, and may eventually be incorporated into future diagnostic criteria. 76 …”

“…Proven VOD/SOS requires histological evidence of disease or documented elevated hepatic venous wedge pressure. 76 Similarly, the Cairo/Cooke diagnostic criteria have also removed the time constraint for VOD/SOS onset, and include ultrasound evidence of VOD/SOS and transfusion-refractory thrombocytopenia as clinical criteria. 80 While the Baltimore and Seattle criteria are the most validated diagnostic tools, the EBMT and Cairo/Cooke criteria are increasingly used in clinical practice, based on the incorporation of more comprehensive, sensitive, and specific criteria for VOD/SOS detection.…”

How to prevent sinusoidal obstruction syndrome/veno-occlusive disease?

“…Additionally, in 2023, the same group divided SOS/VOD into three categories: probable, clinical, and confirmed. Clinical SOS/VOD is diagnosed when, in addition to elevated bilirubin levels (≥ 2 mg/dL), two other clinical SOS/VOD criteria are met (Table 1); it is probable based on less restrictive clinical criteria if the results of ultrasonography (US) and/or elastography confirm the diagnosis, and confirmed if the diagnosis is supported by histopathological or hemodynamic examination (Table 1) [34]. Patients meeting the criteria for probable SOS/ /VOD should be monitored very closely in order to initiate therapeutic intervention as soon as possible, before the occurrence of MOF, which is expected to improve treatment outcomes.…”