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[伴有并存内分泌病的POEMS综合征——病例报告]

[The POEMS syndrome with coexisting endocrinopathy--case report].

作者信息

Lizis-Kolus Katarzyna, Kowalska Aldona, Nowakowska-Domagała Maria

机构信息

Dział Endokrynologii i Medycyny Nuklearnej Swietokrzyskiego Centrum Onkologii, Kielce.

出版信息

Endokrynol Pol. 2007 May-Jun;58(3):238-43.

Abstract

We present a case of a woman with unique multisystem disorder--POEMS syndrome and endocrine abnormalities coexisting with it. The POEMS acronym comprises the dominant features: polyneuropathy, organomegaly, endocrinopathy, monoclonal protein (M protein), skin changes. Association between plasma cell dyscrasia and polyneuropathy, was described in 1956 year by Crow. The main features were coined in the acronym POEMS by Bardwick in 1980 year. The polysymptomatic clinical picture, progressive course and no-concurrent manifestations of main features impede the diagnosis. In this case, the first symptoms were the sensomotor polyneuropathy, peripheral oedema, osteosclerotic bone lesions, skin changes, organomegaly. They preceded diagnosis by 3 years. The first endocrinopathy was hypothyroidism. Definite diagnosis was delayed because we couldn't detect the presence of M protein. Immunoelectrophoresis didn't detect it, but analysis by immunofixation detected M protein in serum and urine. Within 3 years of the first symptoms, she developed hypogonadism hypergonadotropic. At first, the monotherapy with corticosteroids was used, then--melfalan with prednisone. Due to the progression of the disease, a thalidomide was used in therapy (it is anti-VEGF agent). One of the side effects of the treatment of thalidomide is the progression of polyneuropathy, which was observed in this patient. After finishing this therapy she received chemotherapy. This case report imposes the necessity of constants observation of patients with POEMS syndrome because there is a possibility of their developing other disorders. In the event of coexistence polyneuropathy and plasma cell dyscrasia, this disease should be taken into consideration.

摘要

我们报告一例患有独特多系统疾病——POEMS综合征且伴有内分泌异常的女性病例。POEMS这个首字母缩略词包含了主要特征:多发性神经病、脏器肿大、内分泌病、单克隆蛋白(M蛋白)、皮肤改变。1956年Crow描述了浆细胞异常增生与多发性神经病之间的关联。1980年Bardwick将主要特征归纳为首字母缩略词POEMS。多症状的临床表现、病情进展以及主要特征无并发表现都妨碍了诊断。在该病例中,最初的症状是感觉运动性多发性神经病、外周水肿、骨硬化性骨病变、皮肤改变、脏器肿大。这些症状在确诊前3年就已出现。最初的内分泌病是甲状腺功能减退。由于未能检测到M蛋白,明确诊断被推迟。免疫电泳未检测到它,但免疫固定分析在血清和尿液中检测到了M蛋白。在出现最初症状的3年内,她发展为高促性腺激素性性腺功能减退。起初使用皮质类固醇单药治疗,然后是美法仑联合泼尼松。由于疾病进展,治疗中使用了沙利度胺(一种抗血管内皮生长因子药物)。沙利度胺治疗的副作用之一是多发性神经病进展,该患者就出现了这种情况。完成该治疗后她接受了化疗。本病例报告强调了对POEMS综合征患者进行持续观察的必要性,因为他们有可能并发其他疾病。如果存在多发性神经病和浆细胞异常增生同时存在的情况,应考虑这种疾病。

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