Hogan W J, Lacy M Q, Wiseman G A, Fealey R D, Dispenzieri A, Gertz M A
Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA.
Bone Marrow Transplant. 2001 Aug;28(3):305-9. doi: 10.1038/sj.bmt.1703108.
POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) is a plasma cell dyscrasia that differs substantially from classic multiple myeloma. It is often associated with disabling polyneuropathy in younger patients. Current therapeutic approaches are frequently inadequate and leave many patients wheelchair-bound with significant deterioration in quality and length of life. We present the case of a young man with progressive disease despite conventional therapeutic approaches. We describe a novel approach to treatment with a bone-seeking radiopharmaceutical, samarium-153 ethylene diamine tetramethylene phosphonate ((153)Sm-EDTMP), followed by myeloablative chemotherapy with autologous hematopoietic progenitor cell reconstitution. This approach resulted in regression of the organomegaly and skin changes and in neurologic improvement both clinically and electrophysiologically. The patient progressed from being wheelchair-bound to independent ambulation. An aggressive approach should be considered in patients with POEMS syndrome in whom standard therapeutic measures fail.
POEMS综合征(多发性神经病、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变)是一种浆细胞异常增生症,与经典的多发性骨髓瘤有很大不同。它常导致年轻患者出现致残性多发性神经病。目前的治疗方法往往效果不佳,许多患者只能依靠轮椅行动,生活质量和寿命显著下降。我们报告了一例尽管采用了传统治疗方法但病情仍进展的年轻男性病例。我们描述了一种用亲骨性放射性药物钐-153 乙二胺四亚甲基膦酸盐((153)Sm-EDTMP)治疗的新方法,随后进行清髓性化疗并进行自体造血祖细胞重建。这种方法导致器官肿大和皮肤改变消退,临床和电生理方面的神经功能均有改善。患者从依靠轮椅行动恢复到能够独立行走。对于标准治疗措施失败的POEMS综合征患者,应考虑采取积极的治疗方法。