POEMS syndrome is a life-threatening, multi-system disorder driven by an underlying plasma cell neoplasm, characterized by mandatory major criteria such as polyneuropathy and a clonal plasma cell disorder. Diagnosis requires a careful combination of clinical evaluations, radiographic bone assessments, bone marrow biopsies, and elevated vascular endothelial growth factor (VEGF) levels. Because the condition's pathogenesis remains poorly understood, risk stratification relies primarily on the patient's clinical phenotype and specific prognostic indicators like age and renal function. Treatment strategies are generally risk-adapted, utilizing localized irradiation for dominant solitary plasmacytomas or systemic therapy—such as alkylator-based regimens, immunomodulatory drugs, and autologous stem cell transplantation—for disseminated bone marrow involvement. Emphasizing prompt recognition, supportive care, and clone-directed therapies yields the best outcomes for patients managing this complex and rare syndrome.
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