Abstract
POEMS syndrome is a rare paraneoplastic multisystemic disorder caused by an underlying monoclonal plasma-cell dyscrasia and characterized by Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder, and Skin changes. Because early manifestations often overlap with other conditions such as chronic inflammatory demyelinating polyneuropathy (CIDP) and multiple myeloma, diagnosis is frequently delayed.
Here, we describe the case of a 53-year-old women who initially presented with progressive upper-extremity weakness and sensorimotor neuropathy that was diagnosed and treated first as CIDP with IVIG. Her symptoms progressed to include lower extremities weakness requiring wheelchair use along with systemic abnormalities including menorrhagia with iron-deficiency anemia, thrombocytosis, venous thromboembolism and pulmonary embolism (PE). Further evaluation revealed elevated serum free light chains kappa and lambda levels with elevated kappa/lambda ratio, bone marrow biopsy demonstrated elevated plasma cells with flow cytometry consistent with monoclonal plasma cells population kappa-light chain restricted, and markedly elevated plasma vascular endothelial growth factor (VEGF) levels satisfying current diagnostic criteria for POEMS syndrome. Treatment with lenalidomide and dexamethasone resulted in rapid normalization of VEGF and clinical neurologic improvement. Despite sustained biochemical control, the patient developed significant long-term complications including bilateral foot drop, contractures, extremities lymphedema, and progressive restrictive pulmonary dysfunction.
Keywords
POEMS, Polyneuropathy, VEGF, Plasma cell dyscrasia, CIDP, Lenalidomide, Restrictive lung disease, Case report