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POEMS 综合征患者腹水的特征。

The characteristics of ascites in patients with POEMS syndrome.

机构信息

Department of Neurology, Chinese PLA General Hospital, 28 FuXing Road, Beijing, 100853, People's Republic of China.

出版信息

Ann Hematol. 2013 Dec;92(12):1661-4. doi: 10.1007/s00277-013-1829-7. Epub 2013 Jun 28.

Abstract

The characteristics of ascites in patients with POEMS syndrome, which comprise polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes, are unknown. We described the frequency of ascites at presentation of POEMS syndrome and further evaluated for the pathogenesis and nature of the ascites. One hundred and six consecutive patients with POEMS syndrome in Chinese PLA General Hospital were evaluated for the presence of ascites, and the cellular and biochemical characteristics of the ascitic fluids were assessed. Serum levels of complement, cytokines, and clinical chemistry parameters were analyzed in peripheral blood samples of the patients with POEMS syndrome. Ascites was observed in 42 of 106 (39.6 %) patients with POEMS syndrome. Patients with ascites had significantly high serum levels of C3 and C4 complement components and TNF-α (all p < 0.01). In 31 (73.8 %) patients who underwent paracentesis, the ascitic fluids had low serum ascites albumin gradients (SAAG), indicating non-portal hypertension. Spontaneous bacterial peritonitis was not observed. Ascites is a common complication of POEMS syndrome and has characteristics of non-portal hypertension, based on low SAAG. Increased immune activation and inflammatory status could contribute to the pathogenesis of ascites in POEMS syndrome.

摘要

POEMS 综合征患者的腹水特征为多发性神经病、器官肿大、内分泌病、M 蛋白和皮肤改变,但目前尚不清楚。我们描述了 POEMS 综合征患者初次就诊时腹水的发生频率,并进一步评估了腹水的发病机制和性质。对解放军总医院的 106 例连续 POEMS 综合征患者评估了腹水的存在情况,并评估了腹水的细胞和生化特征。分析了 POEMS 综合征患者外周血样本中的补体、细胞因子和临床化学参数水平。在 106 例 POEMS 综合征患者中,有 42 例(39.6%)观察到腹水。有腹水的患者血清补体 C3 和 C4 成分以及 TNF-α 水平显著升高(均 p<0.01)。在 31 例(73.8%)接受腹腔穿刺术的患者中,腹水的血清腹水白蛋白梯度(SAAG)较低,表明非门静脉高压。未观察到自发性细菌性腹膜炎。腹水是 POEMS 综合征的常见并发症,根据低 SAAG,腹水具有非门静脉高压的特征。免疫激活和炎症状态增加可能导致 POEMS 综合征腹水的发病机制。

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