背景:POEMS综合征,一种罕见的副肿瘤疾病,与多个器官有关,多个系统,和多个学科,可能被误认为是其他疾病。因此,诊断经常延迟。在这项工作中,我们研究了POEMS综合征的临床病理特征,以提高早期诊断以防止不可逆转的损害。
方法:我们对9例POEMS进行了临床病理分析,并与相关疾病进行了鉴别诊断。
结果:POEMS综合征患者具有复杂的临床特征,包括周围神经病变,器官肿大,内分泌病,单克隆血浆增殖性疾病,皮肤变化,血管外容量超负荷,硬化骨病变,血小板增多症,和Castleman病.POEMS综合征与其他疾病共享许多要素,区分它们的关键方法是确定是否存在其他基本的POEMS综合征症状或体征。POEMS患者血清和骨髓浆细胞中M蛋白水平低于多发性骨髓瘤(MM)患者。硬化骨病变是POEMS患者的一个显著特点,与MM相比。
结论:POEMS综合征的一些独特的临床病理特征可用于鉴别诊断。这项研究提高了人们对POEMS综合征的认识。
BACKGROUND: POEMS syndrome, a rare paraneoplastic disease, is related to multiple organs, multiple systems, and multiple disciplines and can be mistaken for other disorders. Consequently, the diagnoses are often delayed. In this work we studied the clinicopathologic characteristics of the POEMS syndrome to improve early diagnosis to prevent irreversible damage.
METHODS: We conducted a clinicopathologic analysis of 9 cases of POEMS and made a differential diagnosis with related diseases.
RESULTS: The patients with POEMS syndrome were shown to have complicated clinical characteristics, including peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasma proliferative disorder, skin changes, extravascular volume overload, sclerotic bone lesions, thrombocytosis, and Castleman disease. POEMS syndrome shared many elements with other diseases and the key way to differentiate them was to determine whether there were other fundamental POEMS syndrome symptoms or signs. The level of M-protein in serum and plasma cells in bone marrow of POEMS patients was lower than that of patients with multiple myeloma (MM). Sclerotic bone lesions were a distinctive feature in patients with POEMS, compared with in those with MM.
CONCLUSIONS: Some unique clinicopathologic characteristics of POEMS syndrome can be used for differential diagnosis. This study provides increased awareness of POEMS syndrome.