BLOG: Predicting sinusoidal obstructive syndrome after allogeneic stem cell transplantation
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Sinusoidal obstructive syndrome is a rare but potentially serious complication that can occur after hematopoietic stem cell transplantation.
On average, incidence of sinusoidal obstructive syndrome (SOS) among adults is 10%. In severe cases, the mortality rate can be as high as 80%.
The pathogenesis of SOS after HSCT involves the injury of the sinusoidal endothelial cells of the liver, which leads to loss of wall integrity, endothelial cell detachment, downstream embolization of the centrilobular vein and sinusoidal obstruction. These events result in outflow obstruction, causing hepatic congestion and the development of post-sinusoidal portal hypertension.
Typical clinical features of SOS among adults are hyperbilirubinemia, painful hepatomegaly, ascites and weight gain. In severe cases, SOS can progress to liver failure and multiorgan dysfunction.
Imaging techniques can help detect SOS by showing signs of liver congestion, portal hypertension and parenchymal damage.
Liver stiffness measurement (LSM) is a noninvasive diagnostic tool to assess liver fibrosis and portal hypertension. Several studies have described the role of LSM in diagnosing SOS among patients after allogeneic HSCT.
We showed that LSM after allogeneic HSCT can differentiate between SOS and other causes of bilirubin elevation. Our study included 27 adults. Eleven underwent transient elastography for high SOS risk pre-HSCT. Seventeen underwent transient elastography after HSCT due to bilirubin levels of 2mg/dl or higher with no definite diagnosis of SOS.
The first group had median LSM of 7.4kPa (range, 3.3-22.5). Based on LSM results, the conditioning regimen was modified for six patients.
The second group had median LSM of 7.7kPa (range, 4.4-31.5). Median LSM after HSCT was significantly higher among patients who developed established SOS (n=10) than those who did not (n=8), with values of 10.7kPa (range, 5.6-31.5) vs. 5.9kPa (range, 4.4-13.8), respectively (P=0.02).
We found that an LSM cut-off of 7.5 can predict a diagnosis of SOS with a sensitivity of 80% and a specificity of 86%. However, LSM did not significantly change after HSCT among patients who experienced hepatobiliary complications other than SOS.
Recently, the ElastoVOD study (NCT03426358) conducted in Italy has presented preliminary findings that highlight the effectiveness of LSM in identifying cases of SOS.
A study conducted by Debureaux and colleagues focused on liver stiffness assessment before and after transplantation using transient elastography and 2D-SWE in HSCT patients. The study showed a cut-off value of 8.12kPa using 2D-SWE and 6.87kPa using transient elastography correlated with an SOS diagnosis 14 days after HSCT.
This study showed that baseline LSM did not predict the development of SOS. In this line, Schulz and colleagues reported three cases of fatal SOS among patients who had initially low LSM, but it significantly increased after they developed SOS following transplantation.
However, Colecchia and colleagues demonstrated that patients who developed SOS had numerically higher baseline LSM levels.
Further investigation is needed to determine the impact of LSM assessment and how it can contribute to decision-making prior to HSCT and the development of SOS.
References:
- Bonifazi F, et al. Front Immunol. 2020;doi:10.3389/fimmu.2020.00489.
- Bonifazi F, et al. Transplantation. 2021;doi:10.1097/TP.0000000000003569.
- Carreras E, et al. Bone Marrow Transplant. 2011;doi:10.1038/bmt.2011.65.
- Chan SS, et al. Biol Blood Marrow Transplant. 2020;doi:10.1016/j.bbmt.2020.06.016.
- Colecchia A, et al. Biol Blood Marrow Transplant. 2019;doi:10.1016/j.bbmt.2019.01.019.
- Davidov Y, et al. Bone Marrow Transplant. 2024;doi:10.1038/s41409-024-02288-1.
- de Franchis R, et al. J Hepatol. 2022;doi: 10.1016/j.jhep.2021.12.022.
- Debureaux PE, et al. Haematologica. 2021;doi:10.3324/haematol.2019.245407.
- Lazzari L, et al. Bone Marrow Transplant. 2019;doi:10.1038/s41409-019-0432-5.
- Mohty M, et al. Bone Marrow Transplant. 2023;doi:10.1038/s41409-023-01992-8.
- Ozkan SG, et al. Transfus Apher Sci. 2022;doi:10.1016/j.transci.2022.103370.
- Reddivalla N, et al. Bone Marrow Transplant. 2020;doi:10.1038/s41409-017-0064-6.
- Schulz M, et al. Diagnostics (Basel). 2021;doi:10.3390/diagnostics11060928.
For more information:
Ivetta Danylesko, MD, is an attending physician in the division of hematology and bone marrow transplantation at Sheba Medical Center, as well as a member of the faculty of medicine at Tel Aviv University in Israel. She can be reached at alexivetta@gmail.com.
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