Effective treatment of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes syndrome with congestive heart failure: A case report

BACKGROUND Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome is a rare paraneoplastic syndrome caused by a plasma cell proliferative disorder. The syndrome is characterized by elevated plasma cells, platelets, and vascular endothelial growth factor levels. Although heart disease rarely occurs in POEMS syndrome, the death rate increases sharply after heart failure. We report a patient who initially presented with an endocrine disease and developed congestive heart failure related to POEMS syndrome 9 years later. CASE SUMMARY A 23-year-old woman with no history of menstruation and a 9-year history of type I diabetes reported feeling breathless after activities. She could not lie down and rest at night. Three months prior, she experienced pain and increased tension in her left thigh accompanied by tenderness and edema in both lower extremities. The chief complaint upon hospital admission was that blood sugar has increased for more than 9 years, pain in the left thigh, and edema in both legs for more than 2 mo. After a multisystem evaluation, she was diagnosed with POEMS syndrome. Her echocardiogram showed left ventricular dilation with systolic dysfunction, and the left ventricular ejection fraction was only 38% with severely elevated brain natriuretic peptide. She received a combination of dexamethasone and thalidomide for 1 mo, but her symptoms did not improve. Therefore, we added a two-per-week bortezomib injection. After 2 wk, the patient's heart function had improved significantly. CONCLUSION This case provides information about the treatment of POEMS syndrome with complications and highlights the challenges of developing a standardized treatment.