Laurenti Luca, De Matteis Silvia, Sabatelli Mario, Conte Amelia, Chiusolo Patrizia, Sorà Federica, Piccirillo Nicola, Za Tommaso, Tarnani Michela, Rossi Elena, Leone Giuseppe, Sica Simona
Haematology Institute, Università Cattolica del Sacro Cuore, Rome, Italy.
Leuk Res. 2008 Aug;32(8):1309-12. doi: 10.1016/j.leukres.2007.11.029. Epub 2008 Jan 22.
The acronym POEMS refers to polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes. This disease is progressive and weakening for patients and lead to death generally for neurological problem without therapy. We treated four patients affected by POEMS syndrome with front-line chemotherapy and autologous peripheral blood stem cell transplantation (aPBSCT). After a median follow-up of 40.5 months (range 12-52), all patients are alive with slow but progressive improvement in neurological disease, skin changes, performance status and without evidence of clonal plasmacytosis and organomegaly. In conclusion early diagnosis is crucial to obtain best response and improve clinical outcome.
首字母缩略词POEMS代表多发性神经病、脏器肿大、内分泌病、M蛋白、皮肤改变。这种疾病对患者来说是进行性且会导致身体衰弱的,若不进行治疗,通常会因神经问题导致死亡。我们采用一线化疗和自体外周血干细胞移植(aPBSCT)治疗了4例POEMS综合征患者。经过中位40.5个月(范围12 - 52个月)的随访,所有患者均存活,神经疾病、皮肤改变、体能状态有缓慢但持续的改善,且没有克隆性浆细胞增多症和脏器肿大的迹象。总之,早期诊断对于获得最佳反应和改善临床结局至关重要。