POEMS Syndrome: a case report and review

Abstract

POEMS syndrome is a rare multi-system condition that arises from an underlying plasma cell disorder. We report a case of a 69-year-old lady who presented with symptoms of exertional breathlessness and leg swelling, and a recent history of peripheral neuropathy, borderline diabetes mellitus and monoclonal gammopathy of undetermined significance (MGUS).  The development of worsening right heart failure, which remained refractory despite diuretic therapy, pulmonary hypertension, organomegaly, hypothyroidism, chronic kidney disease, right pleural effusion and ascites, did not lead to a definitive diagnosis until cutaneous abnormalities were recognised.  A presumptive diagnosis of POEMS was subsequently confirmed with the finding of raised serum levels of VEGF and bone marrow histopathology. Our case highlights the complex nature of this multi-system syndrome, the potential for delayed diagnosis and the importance of an integrated sub-specialty approach to management.