POEMS Syndrome Complicated by Portal Hypertension Resembling Decompensated Cirrhosis: A Case Report and Diagnostic Insights

Hua Zhou¹Yangdong Zhou¹Lifang Wu²Ling Yan¹Jie Wei³Weixian Chen^1*Xi Huang^4*Shaocheng Zhang^2*

• ¹Department of Laboratory Medicine, The Second Affiliated Hospital of Chongqing Medical University, Chongqing, 400016, China, Chongqing, China
• ²Department of Laboratory Medicine, The Second Affiliated Hospital of Chengdu Medical College (Nuclear Industry 416 Hospital), Chengdu, China
• ³Department of Clinical Laboratory, Zhuhai People's Hospital (Zhuhai Clinical Medical College of Jinan University), Zhuhai, China., Zhuhai, China
• ⁴Department of Hematology, The Second Affiliated Hospital of Chongqing Medical University, Chongqing, 400016, China, Chongqing, China

A 39-year-old woman presented with progressive fatigue and abdominal distension over six months, accompanied by limb numbness in the last three months. She was initially diagnosed with decompensated cirrhosis at another hospital, with ascites and esophagogastric varices. Symptoms partially improved with diuretic therapy. However, three months later, she developed peripheral neuropathy characterized by "numbness in hands, lower legs, and feet, with a cotton-wool sensation while walking." Further investigations at our hospital revealed IgA-λ type M-protein by immunofixation electrophoresis (IFE), elevated vascular endothelial growth factor (VEGF) (145.96 pg/ml), multiple lymphadenopathies, and endocrine abnormalities (hypothyroidism and menstrual irregularities), leading to a diagnosis of POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin changes). Following chemotherapy with the KPD (carfilzomi, pomalidomide and dexamethasone) regimen and autologous hematopoietic stem cell transplantation (ASCT), the patient showed significant improvement in neurological function and systemic symptoms. This case highlights that after excluding common causes of cirrhosis, such as viral hepatitis, autoimmune liver disease, Wilson's disease, and metabolic dysfunction-associated steatohepatitis (MASH), the patient received repeated symptomatic treatment for cirrhosis. Furthermore, the cirrhotic facies resembled the skin hyperpigmentation of POEMS syndrome, contributing to atypical presentations and diagnostic delay. POEMS syndrome should be suspected in patients with unexplained cirrhosis, ascites, and multi-system damage. Immunofixation electrophoresis for monoclonal protein is crucial for definitive early diagnosis, and VEGF testing also holds certain diagnostic significance.