POEMS综合征的诊断和治疗

Diagnosis and therapy for POEMS syndrome

POEMS综合征是一种罕见的浆细胞疾病,近年来在POEMS的诊断和治疗方面都取得了很大进展。本文将重点阐述POEMS综合征的诊断标准以及早期诊断的要点。在治疗方面,自体造血干细胞移植由于其较好的疗效已经成为年轻POEMS综合征患者的一线治疗。马法兰联合地塞米松也有着较高的缓解率和很好的安全性,可以作为老年或其他不适合移植患者的治疗选择。新药例如来那度胺等给POEMS综合征患者提供了更多的治疗选择。

POEMS syndrome is a rare clonal plasma cell disease characterized by polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes. Significant advances have been made in the diagnosis and treatment of POEMS syndrome over the last decade. In this study, we reviewed the diagnostic criteria and characteristic features of POEMS syndrome. We also focused on the role of the characteristic features of POEMS syndrome in early diagnosis. Autologous peripheral blood stem cell transplantation has become the first-line treatment for younger patients with normal organ function because it has resulted in a high response rate and durable remission. Melphalan and dexamethasone is an effective and well-tolerated treatment for older patients and those with organ dysfunction. Patients with poor performance status or renal function can benefit from novel agents that can also improve transplantation eligibility.