POEMS Syndrome

A patient presented for evaluation of grade 4 optic disc swelling in both eyes. The patient has a history of polyneuropathy both sensory and motor involving the upper limbs more than lower limbs consistent with a diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) and received a 6-month course of IVIG with no improvement. Lab analysis revealed high serum Immunoglobulin G and an M-spike on electrophoresis. His lumbar puncture revealed a normal opening pressure and a high IGG index. Brain and orbital MRIs were unremarkable. The constellation of polyneuropathy, gammopathy, and optic disc swelling raised the suspicion of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes) syndrome. The patient was referred to hematology where complete blood count (CBC) showed an elevated platelet count. A bone marrow biopsy showed >20% plasma cells consistent with multiple myeloma confirming the diagnosis of POEMS. The patient was given bortezomib and dexamethasone. Two months later the patient showed significant improvement in optic disc swelling, polyneuropathy, and immunoglobulin count. This case shows how a common finding can be the clue to diagnosing an uncommon condition.

Presentation Date: 03/31/2022

Issue Date: 04/08/2022