Leave It to the Prose: A Case of POEMS Syndrome

POEMS syndrome is a rare paraneoplastic syndrome named for its constellation of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. As a proliferative plasma cell disorder, it has a multisystem presentation and chronic cytokine overproduction. It often presents in the fifth or sixth decade of life, mostly in males. Presentations vary widely, making it extremely difficult to diagnose. A chronic progressive distal, sensorimotor polyneuropathy in conjunction with a monoclonal plasma cell dyscrasia suggests POEMS. A sixty-one-year-old man with a significant past medical history of bilateral lower extremity weakness and a cerebral meningioma presented with anorexia, intermittent dysphagia, and early satiety with intermittent vomiting and diarrhea over the past three months with a 20-pound weight loss. A CT of the abdomen and pelvis was obtained due to vomiting, showing expansile lesions of the left ischium, acetabulum, and ilium, with small-volume ascites and splenomegaly. Because of these findings and the noted bilateral lower extremity neuropathic weakness, we obtained further testing to corroborate suspicions of a plasma cell disorder. Labs were significant for IgG lambda with a monoclonal spike (M spike), low cortisol, low testosterone, and elevated thyroid-stimulating hormone (TSH). Protein electrophoresis was positive for bi-clonal lambda, and his vascular endothelial growth factor (VEGF) levels were elevated to 377. Bone marrow core biopsy indicated malignancy. Given his polyneuropathy, organomegaly, endocrinopathy, IgG lambda, skin changes, and extravascular fluid overload, POEMS syndrome was diagnosed. He was initiated on lenalidomide and dexamethasone and eventually received a stem cell autologous bone marrow transplant. Given his non-specific presentation, and the confounding factor of a known meningioma causing hypopituitarism, diagnosing POEMS required extensive workup. A contrast CT demonstrating bone lesions associated with myeloma-type disease was crucial, pairing his endocrinopathy and neuropathy with a plasma cell dyscrasia. Although POEMS is exceedingly rare, accurate diagnosis is vital, as treatment requires a multidisciplinary approach. While high-dose chemotherapy-conditioned autologous stem cell transplantation is the gold-standard treatment for POEMS syndrome, patients who are diagnosed in a late stage of the disease are not candidates, underlining the need for early identification of the disorder.