POEMS Syndrome: 2026 Update on Diagnosis, Risk-Stratification, and Management.

Area of Science:

• Hematology
• Oncology
• Neurology

Background:

• POEMS syndrome is a rare, life-threatening condition linked to plasma cell neoplasms.
• Key diagnostic criteria include polyneuropathy, clonal plasma cell disorder, sclerotic bone lesions, elevated VEGF, and Castleman disease.
• Minor features encompass organomegaly, endocrinopathy, skin changes, papilledema, volume overload, and thrombocytosis.

Purpose of the Study:

• To outline the diagnostic criteria for POEMS syndrome.
• To discuss risk stratification based on clinical phenotype.
• To review current risk-adapted therapeutic strategies for POEMS syndrome.

Main Methods:

• Diagnosis relies on meeting three major criteria (including polyneuropathy and clonal plasma cell disorder) and at least one minor criterion.
• Risk stratification considers clinical factors like low albumin, age, pleural effusion, pulmonary hypertension, and reduced eGFR.
• Therapeutic approaches involve irradiation for dominant plasmacytoma and systemic therapies for diffuse disease, with agents like alkylators, lenalidomide, thalidomide, and bortezomib.

Main Results:

• The diagnosis of POEMS syndrome is established by a specific combination of major and minor criteria.
• Clinical phenotype, rather than molecular markers, informs risk stratification due to poorly understood pathogenesis.
• Various treatment modalities exist, including irradiation, systemic chemotherapy, and targeted agents, with daratumumab combinations showing promise.

Conclusions:

• Early recognition and prompt initiation of supportive care and plasma cell-directed therapy are crucial for optimal POEMS syndrome outcomes.
• Risk-adapted therapy, guided by clinical assessment, is essential for managing this complex syndrome.
• Ongoing research into pathogenesis may lead to improved risk stratification and targeted treatments.