POEMS综合征的内分泌表现及预后分析

Endocrinological manifestations and prognosis of POEMS syndrome

目的 提高对POEMS综合征内分泌表现的认识 ,以进一步研究其发病机制和治疗方案。方法 分析 85例POEMS综合征患者内分泌改变的临床表现和实验室资料 ,总结其治疗方案和治疗效果。结果 85例中 ,男性勃起功能障碍 4 6例 ,男性乳房发育 2 5例 ,女性闭经 11例 ,皮肤色素沉着 6 7例 ,皮肤多毛 31例 ,甲状腺功能减退 12例 ,糖耐量异常 16例 ,肾上腺皮质功能减退 1例。应用皮质激素治疗后 ,临床症状可短暂缓解 ,减量或停用时易复发 ;2例患者改用雄激素治疗和 2例试用三苯氧胺治疗后 ,临床症状改善明显 ,且随访无复发。结论 本病好发于中年男性。内分泌紊乱多表现为性功能低下 ,其次为甲状腺功能低下及糖耐量异常 ,肾上腺功能低下少见。三苯氧胺或雄激素等抗雌激素药物治疗POEMS综合征的疗效明确 ,且复发少。

Objective To raise the awareness of endocrinological manifestations of POEMS syndrome. Methods An analysis of clinical picture and laboratory examination was summerized in 85 cases of POEMS syndrome occurred in this country, and the therapeutic regime and effectiveness were also evaluated.Results Among the 85 cases ,there were 46 erectile dysfunction, 25 gynecomastia, 11 amenorrhea, 67 cutaneous pigmentation, 31 pilosity, 12 hypothyroidism, 16 glucose intolerance, and 1 hypoadrenocortical function.Conclusions POEMS syndrome usually occurs in middle-age males with most frequent endocrinological manifestations presenting as erectile dysfunction, whereas hypothyroidism and glucose intolerance are the next and only rarely as hypoadrenalism. Tamoxifen and androgen are obviously the effective therapy with low recurrence.