Treatment strategy for POEMS syndrome

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) is a paraneoplastic disease associated with an underlying plasma cell dyscrasia. This syndrome usually progresses slowly and can be life-threatening. No large-scale controlled study providing convincing evidence for the treatment of this rare condition has thus for been conducted. However, several lines of evidence have shown the efficacy of conventional therapies such as radiation, alkylator-based treatment, and corticosteroid therapy. Recent studies have also proposed novel therapeutic strategies such as autologous peripheral blood stem cell transplantation (auto-PBSCT) and thalidomide, lenalidomide, and bevacizumab therapies. We reviewed the current treatment strategies for POEMS syndrome and summarized them as follows; 1) Radiotherapy or surgical resection is highly effective for isolated plasmacytoma. 2) Melphalan-predonisolone administration may be a treatment option for the patients with widespread bone lesions. 3) High dose chemotherapy with auto-PBSCT is a promising novel approach that can lead to dramatic improvement particularly in patients with good systemic condition. 4) Plasma exchange and immunoglobulin treatment are not recommended. 5) Administration of thalidomide and lenalidomide may ameliorate the clinical conditions of the patients who are unable to undergo auto-PBSCT; however, this needs further confirmation. Finally, we emphasized that the efficacy of these therapies largely depends on the appropriate early diagnosis of POEMS syndrome.