Balcar Lorenz, Degenfeld-Schonburg Lina, Hopp Can, Kramer Georg, Graf Irene, Simonis Lucie, Sillaber Christian, Kalteis Stephanie, Gisslinger Heinz, Meischl Tobias, Stättermayer Albert Friedrich, Mandorfer Mattias, Mitterbauer-Hohendanner Gerlinde, Reiberger Thomas, Trauner Michael, Scheiner Bernhard, Krauth Maria-Theresa, Semmler Georg
Division of Gastroenterology and Hepatology, Department of Medicine III, Medical University of Vienna, Vienna, Austria.
Vienna Hepatic Hemodynamic Laboratory, Division of Gastroenterology and Hepatology, Department of Medicine III, Medical University of Vienna, Vienna, Austria.
Liver Int. 2025 Jun;45(6):e70098. doi: 10.1111/liv.70098.
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.
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.
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.
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.
BCR::ABL1阴性骨髓增殖性肿瘤（MPN）病程常因内脏静脉系统血栓栓塞事件而复杂化，导致门静脉高压（PH）。因此，引入脾脏硬度测量（SSM）可能会改善PH的诊断。本研究的目的是评估SSM（使用100Hz探头进行）在这些患者PH无创分层中的临床应用价值。
我们进行了一项回顾性、单中心横断面分析，纳入了2023年10月至2024年9月期间在维也纳医科大学血液科门诊就诊、有可用肝脏硬度（LSM）/SSM数据的连续性BCR::ABL1阴性MPN患者。LSM/SSM与PH的体征和事件相关联。
纳入55例患者（平均年龄57.9±14.2岁，69%为女性，以真性红细胞增多症为主）。四分之一的患者有内脏静脉血栓形成。19例患者（34.5%）有PH的特异性体征，29例患者（52.7%）有非特异性体征。12例患者（21.8%）在纳入研究前发生过PH事件。SSM与疾病严重程度相关（即JAK2 V617F等位基因频率）。LSM/SSM能充分区分有PH和无PH的患者。虽然SSM与脾肿大密切相关，但它在脾肿大之外还能提供关于PH的独立信息。采用序贯LSM（<5和≥15kPa）和SSM（<21和≥50kPa）来判断PH的存在与否，减少了灰色区域（24%），且具有足够的敏感性/特异性。
虽然SSM与脾肿大和疾病严重程度密切相关，但在BCR::ABL1阴性MPN患者中，它与PH独立相关。实施LSM/SSM可能会改善患者管理。
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