Idiopathic portal hypertension associated with POEMS syndrome mimicking liver cirrhosis in a patient with chronic HBV infection

Abstract

AbstractPolyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes, known as POEMS syndrome, is a rare paraneoplastic syndrome resulting from an underlying plasma cell disorder. In contrast to the common presentations such as peripheral neuropathy, skin changes, extravascular fluid overload, and endocrinopathy, POEMS syndrome presenting as idiopathic portal hypertension (IPH) has been reported in only seven cases. A 58‐year‐old man diagnosed as hepatitis B virus (HBV)‐related cirrhosis in another hospital presented to our outpatient clinic with tense ascites. Initial findings of laboratory examinations, ultrasound, and magnetic resonance imaging suggested the diagnosis of cirrhosis with ascites and hepatorenal syndrome. The patient's renal function improved after terlipressin administration but deteriorated once it was withdrawn. A hepatic venous pressure gradient was measured as 10 mmHg. The patient was implanted with a transjugular intrahepatic portosystemic shunt (TIPS) and achieved better renal function and ascites control. Even though this scenario was consistent with a diagnosis of cirrhosis‐related portal hypertension, a low serum‐ascites albumin gradient (SAAG) and liver stiffness value prompted further studies. The presence of peripheral polyneuropathy, monoclonal gammopathy, elevated vascular endothelial growth factor levels, and endocrinopathies resulted in a diagnosis of POEMS syndrome. The patient received prednisolone along with low‐dose diuretics and achieved good control of his ascites. A percutaneous liver biopsy showed focal enlarged portal tracts with fibrosis, small lymphocyte infiltration, and absence of cirrhosis, supporting a diagnosis of IPH. This is a report of a rare case of IPH associated with POEMS syndrome mimicking cirrhosis. Our management of this patient also highlights the usefulness of the SAAG and a liver stiffness value to arrive at the proper diagnosis and furthermore provides an opportunity to learn from the experience of combination treatment with prednisolone and TIPS.