Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2025 Jun;45(6):e70098.
doi: 10.1111/liv.70098.

Non-Invasive Stratification of Portal Hypertension in Patients With BCR::ABL1-Negative Myeloproliferative Neoplasms

Lorenz Balcar  1   2   3 , Lina Degenfeld-Schonburg  4 , Can Hopp  1   2   3 , Georg Kramer  1   2   3 , Irene Graf  4 , Lucie Simonis  1   2   3 , Christian Sillaber  4 , Stephanie Kalteis  1   2   3 , Heinz Gisslinger  4 , Tobias Meischl  1   5 , Albert Friedrich Stättermayer  1   2 , Mattias Mandorfer  1   2   3 , Gerlinde Mitterbauer-Hohendanner  6 , Thomas Reiberger  1   2   3 , Michael Trauner  1   2   3 , Bernhard Scheiner  1   2   3 , Maria-Theresa Krauth  4 , Georg Semmler  1   2   3
Affiliations

Non-Invasive Stratification of Portal Hypertension in Patients With BCR::ABL1-Negative Myeloproliferative Neoplasms

Lorenz Balcar et al. Liver Int. 2025 Jun.

Abstract

Background & aims: The course of BCR::ABL1-negative myeloproliferative neoplasms (MPN) is frequently complicated by thromboembolic events in the splanchnic venous system, resulting in portal hypertension (PH). Therefore, the introduction of spleen stiffness measurement (SSM) might improve the diagnosis of PH. The aim of this study was to evaluate the clinical utility of SSM (performed by using the 100 Hz probe) in non-invasive stratification of PH in these patients.

Methods: We performed a retrospective, monocentric, cross-sectional analysis including consecutive patients with BCR::ABL1-negative MPN attending the haematological outpatient clinic at the Medical University of Vienna with available liver stiffness (LSM)/SSM from 10/2023 to 09/2024. LSM/SSM were linked to signs and events of PH.

Results: Fifty-five patients were included (mean age 57.9 ± 14.2 years, 69% females, polycythaemia vera as main entity). One fourth of patients had splanchnic vein thrombi. Nineteen patients (34.5%) had specific and 29 patients (52.7%) had unspecific signs of PH. Twelve patients (21.8%) experienced PH events prior to study inclusion. SSM correlated with disease severity (i.e., JAK2 V617F allele frequency). LSM/SSM adequately stratified patients with vs. without PH. While SSM was strongly linked with splenomegaly, it yielded independent information regarding PH on top of splenomegaly. The implementation of sequential LSM (< 5 & ≥ 15 kPa) and SSM (< 21 & ≥ 50 kPa) for ruling in and out PH reduced the grey zone (24%) with adequate sensitivity/specificity.

Conclusions: While SSM is strongly correlated with splenomegaly and disease severity, it is independently associated with PH in patients with BCR::ABL1-negative MPN. Implementation of LSM/SSM might improve patient management.

Keywords: MPN; PSVD; SSM; portal hypertension; spleen stiffness.

PubMed Disclaimer

Conflict of interest statement

Tobias Meischl received speaker honoraria from AstraZeneca, Chiesi, Janssen‐Cilag, Novartis, and sobi; consulting fees from CSL Behring; and travel support from AstraZeneca, BeiGene, CSL Behring, Chiesi, Gilead, Jazz Pharmaceuticals, Janssen‐Cilag, Novartis, and teva‐ratiopharm. Mattias Mandorfer served as a speaker and/or consultant and/or advisory board member for AbbVie, Gilead, Collective Acumen, and W. L. Gore & Associates, Takeda and received travel support from AbbVie, Bristol‐Myers Squibb, and Gilead. Thomas Reiberger served as a speaker and/or consultant and/or advisory board member for AbbVie, Bayer, Boehringer Ingelheim, Gilead, Intercept, MSD, Roche, Siemens, and W. L. Gore & Associates and received grants/research support from AbbVie, Boehringer Ingelheim, Gilead, MSD, Philips, and W. L. Gore & Associates as well as travel support from Boehringer Ingelheim and Gilead. Michael Trauner received speaker fees from BMS, Falk Foundation, Gilead, Intercept, Ipsen, Janssen, Madrigal, MSD, and Roche; he advised for Agomab, AbbVie, Albireo, BiomX, Boehringer Ingelheim, Chemomab, Cymabay, Falk Pharma GmbH, Genfit, Gilead, Hightide, Intercept, Ipsen, Janssen, Mirum, MSD, Novartis, Phenex, Pliant, Rectify, Regulus, Siemens, and Shire. He further received travel support from AbbVie, Falk, Gilead, Intercept, and Janssen and research grants from Albireo, Alnylam, Cymabay, Falk, Gilead, Intercept, MSD, Takeda, and UltraGenyx. He is also a co‐inventor of patents on the medical use of norUDCA filed by the Medical Universities of Graz and Vienna. Bernhard Scheiner received travel support from AbbVie, Ipsen, and Gilead. Georg Semmler received travel support from Amgen. The other authors declare no conflicts of interest.

Figures

FIGURE 1
FIGURE 1
Comparison of LSM and SSM in healthy controls (HC), MPN patients without signs of portal hypertension (PH), as well as patients with unspecific and specific signs of PH. HC, healthy controls; PH, portal hypertension.
FIGURE 2
FIGURE 2
Comparison of LSM, SSM, and SSM‐to‐LSM ratio according to presence/absence of specific signs of portal hypertension (A–C), unspecific signs of portal hypertension (D–F) and portal hypertensive events (G–I). LSM, liver stiffness measurement; SSM, spleen stiffness measurement.
FIGURE 3
FIGURE 3
Receiver operating characteristic curves for specific signs of portal hypertension (A), unspecific signs of portal hypertension (B), and portal hypertensive events (C). LSM, liver stiffness measurement; PLT, platelet count; SSM, spleen stiffness measurement.
FIGURE 4
FIGURE 4
Scatterplot of SSM and spleen diameter. The red line represents the linear fit; and the yellow line represents a non‐linear fit with natural splines and two degrees of freedom (df) for (more) flexible modelling of the relationship between SSM and the spleen diameter. SSM, spleen stiffness measurement.
FIGURE 5
FIGURE 5
Performance of cut‐offs for ruling in and ruling out specific signs of portal hypertension/portal hypertensive events of LSM and SSM in the study cohort. LSM, liver stiffness measurement; NPV, negative predictive value; PH, portal hypertension; PPV, positive predictive value; SSM, spleen stiffness measurement.
See this image and copyright information in PMC

References

    1. Spivak J. L., “Myeloproliferative Neoplasms,” New England Journal of Medicine 376, no. 22 (2017): 2168–2181. - PubMed
    1. Smalberg J. H., Arends L. R., Valla D. C., Kiladjian J. J., Janssen H. L. A., and Leebeek F. W. G., “Myeloproliferative Neoplasms in Budd‐Chiari Syndrome and Portal Vein Thrombosis: A Meta‐Analysis,” Blood 120, no. 25 (2012): 4921–4928. - PubMed
    1. Hoekstra J., Bresser E. L., Smalberg J. H., Spaander M. C., Leebeek F. W., and Janssen H. L., “Long‐Term Follow‐Up of Patients With Portal Vein Thrombosis and Myeloproliferative Neoplasms,” Journal of Thrombosis and Haemostasis 9, no. 11 (2011): 2208–2214. - PubMed
    1. Elkrief L., Payancé A., Plessier A., et al., “Management of Splanchnic Vein Thrombosis,” JHEP Reports: Innovation in Hepatology 5, no. 4 (2023): 100667. - PMC - PubMed
    1. Singh S., Muir A. J., Dieterich D. T., and Falck‐Ytter Y. T., “American Gastroenterological Association Institute Technical Review on the Role of Elastography in Chronic Liver Diseases,” Gastroenterology 152, no. 6 (2017): 1544–1577. - PubMed

Substances

LinkOut - more resources