Underdiagnosed VOD: A major cause of multi-organ failure in leukaemia transplant patients

Veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is widely underdiagnosed, due to concomitant comorbidities and multifactorial deterioration, often leading to the vague diagnosis of multi-organ failure. Bazarbachi AH, et al. (2021) analysed a large sample from the EBMT registry, consisting of allogeneic hematopoietic cell transplantation (alloHCT) patients who were reported to have died of multi-organ failure, and determined their exact cause of death focusing on VOD/SOD.

The study utilised a retrospective, registry-based, multicentre analysis of adult patients with acute leukemia who received alloHCT between 2010 and 2018 with the reported cause of death being multi-organ failure anytime post-transplant. Patient-related, transplant-related, graft-versus-host disease-related, and study-specific variables were collected to identify risk factors for VOD/SOS development and assess the proportion of patients meeting VOD/SOS diagnostic criteria.

Key findings

1. High incidence of underdiagnosed VOD/SOS:
   • The study revealed that VOD/SOS is frequently underdiagnosed in acute leukemia transplant patients. Many cases were only identified retrospectively, indicating a significant gap in real-time diagnosis. This underdiagnosis contributes to delayed treatment and higher rates of complications.
2. Correlation with multi-organ failure:
   
   • A strong correlation was found between underdiagnosed VOD/SOS and the occurrence of multi-organ failure. Patients with undiagnosed or late-diagnosed VOD/SOS exhibited higher rates of kidney, lung, and cardiovascular failures, leading to increased mortality.
3. Ineffective diagnostic criteria:
   
   • The analysis highlighted the inadequacy of current diagnostic criteria in capturing all VOD/SOS cases. Many centres relied on outdated or inconsistent criteria, resulting in missed diagnoses. The study advocates for the adoption of refined and standardised diagnostic guidelines, such as those recently proposed by the EBMT.
4. Need for early intervention:
   
   • Early diagnosis and timely intervention were associated with significantly better outcomes. Patients diagnosed early and treated promptly with defibrotide, a key therapeutic agent for VOD/SOS, showed improved survival rates and reduced organ damage.

The underdiagnosis of VOD/SOS is a major contributor to multi-organ failure and mortality in acute leukemia transplant patients. A more precise categorisation of cause-specific death from multi-organ failure will allow for a better understanding of VOD/SOS.

Read the full published article:
Bazarbachi AH, et al. Underdiagnosed veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) as a major cause of multi-organ failure in acute leukemia transplant patients: an analysis from the EBMT Acute Leukemia Working Party. Bone Marrow Transplant. 2021;56:917–927. https://doi.org/10.1038/s41409-020-01135-3.



Posted on 17 July 202417 July 2024