[Treatment strategy for POEMS syndrome]

Area of Science:

• Hematology
• Oncology
• Neurology

Context:

• POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes) is a rare paraneoplastic syndrome linked to plasma cell dyscrasia.
• The condition typically progresses slowly and can be life-threatening, with limited large-scale controlled treatment studies available.
• Current understanding highlights the need for effective management strategies for this complex disorder.

Purpose:

• To review and summarize current and emerging treatment strategies for POEMS syndrome.
• To evaluate the efficacy of various therapeutic approaches, including conventional and novel therapies.
• To emphasize the importance of early diagnosis in optimizing treatment outcomes.

Summary:

• Radiotherapy or surgery is effective for localized plasmacytoma.
• Melphalan-prednisolone may benefit patients with extensive bone disease.
• High-dose chemotherapy with autologous peripheral blood stem cell transplantation (auto-PBSCT) shows promise for suitable candidates.
• Thalidomide and lenalidomide may offer benefits for patients unable to undergo auto-PBSCT, requiring further validation.
• Plasma exchange and immunoglobulin treatments are not recommended.
• Early and accurate diagnosis is paramount for successful POEMS syndrome management.

Impact:

• Provides a comprehensive overview of POEMS syndrome treatment options.
• Informs clinical decision-making for managing this rare condition.
• Highlights the potential of novel therapies like auto-PBSCT and immunomodulatory drugs.
• Underscores the critical role of timely diagnosis in improving patient prognosis.