POEMS综合征患者内分泌改变特点及临床意义

Endocrinopathy in Patients with POEMS Syndrome and Efficacy of Chemotherapy

[目的]探讨POEMS综合征患者内分泌异常的特点、治疗后的变化规律及其临床意义。[方法]收集收治的14例初治POEMS综合征患者（男性9例,女性5例,中位年龄49岁）的临床资料,回顾性分析不同化疗方案对患者内分泌改变的影响。[结果]所有患者均具有内分泌异常,其中毛发增多5例、皮肤色素沉着13例、甲状腺功能异常8例、血糖异常7例、皮质醇节律异常4例。9例男性患者中,出现性功能异常8例、乳腺发育5例。经过化疗后,甲状腺功能、性激素水平和皮质醇节律恢复较早,最快者经过2个疗程即可恢复正常;经过2个疗程后即恢复正常比例最高的是甲状腺功能和皮质醇节律。与非硼替佐米组相比,应用硼替佐米化疗患者的性激素水平恢复快,但差异无统计学意义（P=0.486）;在性激素中,硼替佐米对雌二醇水平恢复作用最为明显。[结论] POEMS综合征内分泌改变涉及到多个内分泌轴,化疗可以有效改善患者的内分泌异常;含硼替佐米的化疗方案相较于传统的化疗方案,可以更有效的改善患者的内分泌异常。

[Objective] To examine the clinical characteristics and efficacy of chemotherapy for endocrinopathy in patients with POEMS（polyneuropathy,organomegaly,endocrinopathy,monoclonal plasma cell-proliferative disorder,skin changes） syndrome. [Methods] The clinical data of14 patients with POEMS syndrome（9 males,5 females,median age 49 years） were retrospectively reviewed,and the changes of endocrinopathy after treatment were analyzed. [Results] There was endocrinopathy in all 14 patients. Five patients had hypertrichosis,13 patients had hyperpigmentation,8 patients had thyroid axis abnormality,7 patients had diabetes or abnormal blood glucose,4 patients had adrenal axis abnormality. Among 9 male patients,8 had erectile dysfunction and 5 had gynecomastia. After chemotherapy,thyroid function,sex hormone level and cortisol rhythm were recovered early. The highest percentage of recovery after 2 cycles of treatment was thyroid function and cortisol rhythm. There was no significant difference in sex hormone recover between patients treated with Bortizemib and those with other drugs（P=0.486）. In the sex hormone recovery,Bortizemib was more effective for estradiol. [Conclusion] POEMS syndrome is an infrequent syndrome with multisystem abnormal. Chemotherapy is effective in improving the abnormal of endocrinopathy,but no significant difference in efficacy was observed between patients treated with Bortezomib and non-Bortizemib.