D'Alitto Felicia, Scherz Amina, Margini Cristina, Von Tengg-Kobligk Hendrik, Montani Matteo, Pabst Thomas, Berzigotti Annalisa
Swiss Liver Center, Hepatology, University Clinic for Visceral Surgery and Medicine, Inselspital, University of Bern, Switzerland.
Department of Medical Oncology, Inselspital, University of Bern, Switzerland.
Cureus. 2018 Jun 8;10(6):e2768. doi: 10.7759/cureus.2768.
Portal hypertension (PH) is a common clinical syndrome leading to severe complications. In the western world, about 90% of cases of PH are due to liver cirrhosis, and thanks to the availability of ultrasound elastography methods, this diagnosis is usually confirmed at bedside. We report a case of a patient presenting with PH and ascites initially suspected of suffering from liver cirrhosis. The finding of large hepatomegaly and a massive increase in liver stiffness prompted us to perform a liver biopsy. This revealed no fibrosis, but diffuse primary amyloidosis (AL amyloidosis). We discuss the diagnostic and treatment of this case, with emphasis on non-invasive imaging methods available for diagnosis and follow up.
门静脉高压（PH）是一种常见的临床综合征，可导致严重并发症。在西方世界，约90%的PH病例归因于肝硬化，并且由于超声弹性成像方法的应用，这种诊断通常可在床边得到证实。我们报告一例表现为PH和腹水的患者，最初怀疑患有肝硬化。肝脏肿大及肝脏硬度大幅增加促使我们进行肝活检。结果显示无纤维化，但为弥漫性原发性淀粉样变性（AL淀粉样变性）。我们讨论该病例的诊断和治疗，重点关注可用于诊断和随访的非侵入性成像方法。
