Measuring spleen stiffness may enhance the diagnosis of portal hypertension in individuals with BCR::ABL1-negative myeloproliferative neoplasms (MPNs), according to research published in liver International. The study highlights a potential noninvasive approach to identify this frequent complication of MPNs.

Splanchnic vein thrombosis (SVT) is a common issue for patients with BCR::ABL1-negative mpns, significantly increasing morbidity and mortality if not addressed, according to Maria-Theresa Krauth, MD, of the medical University of Vienna, and colleagues. They noted,”Conversely,in patients with non-cirrhotic/non-malignant SVT,BCR::ALB1-negative MPN are the most common etiological factors.”

While splenomegaly is often observed in people with BCR::ABL1-negative MPN, the assessment of underlying portal hypertension “can be conflicting and is not standardized,” the authors stated.

vibration-controlled transient elastography (VCTE) has emerged as an effective method for diagnosing portal hypertension in esophageal varices among patients with chronic liver disease. The researchers proposed that this tool could similarly aid in identifying portal hypertension beyond splenomegaly in those with BCR::ABL1-negative MPN.

The authors wrote, “In contrast to liver stiffness measurement (LSM) as an established surrogate of liver fibrosis and associated sinusoidal portal hypertension, SSM offers the unique advantage to not only capture hepatic resistance, but may also capture dynamic (pre-)sinusoidal vasoconstriction and congestion of the portal blood inflow (a potential surrogate of portal hypertension-associated splanchnic hypoperfusion) and portal hypertension-induced splenic fibrosis.”

To investigate this, the team conducted a retrospective study involving 55 patients with BCR::ABL1-negative MPNs treated at the Medical University of Vienna. Participants had undergone LSM/SMM between October 2023 and september 2024.

The majority of participants were female (69%), with polycythemia vera (40%) being the most prevalent MPN, followed by essential thrombocythemia (36.4%) and primary myelofibrosis (20%). The cohort’s average age was 57.9 years. A quarter of the patients (26%) had splanchnic vein thrombi, 34.5% showed specific signs of portal hypertension, and 52.7% exhibited nonspecific signs. Additionally, 21.8% had prior portal hypertension events.

The authors noted, “Compared to the literature, these numbers are high and underline the need for multidisciplinary management of these patients.”

The study found that spleen stiffness measurement (SSM) correlated with disease severity and effectively distinguished between patients with and without portal hypertension. SSM was closely associated with splenomegaly and provided unique insights into portal hypertension, demonstrating the best discrimination. The researchers suggested that a collaborative approach involving hematology and hepatology could optimize patient care, with sequential LSM and SSM reducing diagnostic uncertainty.

“While SSM yielded the best discrimination regarding portal hypertension, the sequential submission of already established, easy-to-remember LSM and SSM cut-offs can adequately rule in/rule out portal hypertension in the majority of patients,” they explained.

The investigators acknowledged the study’s limitations, including its retrospective design and limited sample size. they also noted that while LSM and SSM are “user-amiable,” VCTE may not be universally accessible.

Key Findings on Spleen Stiffness and Portal Hypertension

“Compared to the literature, these numbers are high and underline the need for multidisciplinary management of these patients.”

Researchers at the Medical University of Vienna conducted a study to determine whether spleen stiffness measurements could help improve the diagnosis of portal hypertension in patients with BCR::ABL1-negative myeloproliferative neoplasms (MPNs).The study involved 55 patients who underwent liver and spleen stiffness measurements. The results indicated that spleen stiffness measurement (SSM) correlated with disease severity and effectively distinguished between patients with and without portal hypertension.