Cao Chuang-Jie, Dou Cheng-Yun, Zhou Ke-Hua, Liu Jin-Bo, Lai Hong
Department of Pathology, The First Affiliated Hospital of Sun Yat-san University, Guangzhou, Guangdong 510080, P.R. China.
Department of Hepatology, Qilu Hospital of Shandong University, Jinan, Shandong 250012, P.R. China.
Oncol Lett. 2017 Nov;14(5):5197-5202. doi: 10.3892/ol.2017.6904. Epub 2017 Sep 6.
Polyneuropathy, organomegaly, endocrinopathy, M proteins, and skin changes (POEMS) syndrome is a rare variant of plasma cell disorders with multiple systemic manifestations. A 50-year-old female patient presented with progressive weakness in her upper and lower limbs; tingling, numbness and burning in her feet; polyneuropathy (demyelinating in the majority of cases of POEMS syndrome); monoclonal plasma cell disorder (typicallyλ-restricted in cases of POEMS syndrome); sclerotic lesions on the spine and pelvis; organomegaly, including hepatomegaly, splenomegaly and lymphadenopathy; edema; pleural effusion; adrenal, thyroidal, pituitary, gonadal and pancreatic endocrinopathy; skin changes, including hyperpigmentation, dry skin and hypertrichosis; thrombocytosis; pulmonary hypertension; low vitamin B and weight loss. Following the diagnosis of POEMS syndrome, the patient was treated only with pain-alleviating corticosteroids. Respiratory failure-induced mortality occurred 24 months after the patient first experienced difficulty walking and numbness in her lower extremities. The present study suggests that abnormal symptoms in cases of POEMS syndrome should be further evaluated during the diagnosis and treatment.
多神经病、器官肿大、内分泌病、M蛋白和皮肤改变(POEMS)综合征是一种具有多种全身表现的罕见浆细胞疾病变体。一名50岁女性患者出现上肢和下肢进行性无力;足部刺痛、麻木和烧灼感;多神经病(在大多数POEMS综合征病例中为脱髓鞘性);单克隆浆细胞疾病(在POEMS综合征病例中通常为λ限制型);脊柱和骨盆硬化性病变;器官肿大,包括肝肿大、脾肿大和淋巴结病;水肿;胸腔积液;肾上腺、甲状腺、垂体、性腺和胰腺内分泌病;皮肤改变,包括色素沉着、皮肤干燥和多毛症;血小板增多症;肺动脉高压;维生素B水平低和体重减轻。在诊断为POEMS综合征后,该患者仅接受了缓解疼痛的皮质类固醇治疗。在患者首次出现行走困难和下肢麻木24个月后,因呼吸衰竭死亡。本研究表明,在POEMS综合征病例的诊断和治疗过程中,应进一步评估异常症状。