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Abstract：
BACKGROUND: Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome is a rare paraneoplastic syndrome that encompass multiple systems. The most common clinical symptoms of POEMS syndrome are progressive sensorimotor polyneuropathy, organ enlargement, endocrine disorders, darkening skin, a monoclonal plasma cell proliferative disorder, and lymph node hyperplasia. The organomegaly consists of hepatosplenomegaly and/or lymphadenopathy; cases of cardiomyopathy are rare. Diagnoses are often delayed because of the atypical nature of the syndrome, exposing patients to possibly severe disability. Therefore, identifying atypical symptoms can improve the prognosis and quality of life among POEMS syndrome patients.
METHODS: Herein, we report the case of a 59-year-old woman with POEMS syndrome that involved dilated cardiomyopathy. The patient presented to the hospital with complaints of shortness of breath and discomfort in the chest. The patient reported previous experiences of limb numbness. During hospitalization, the brain natriuretic peptide levels were 3504.0 pg/mL. Color doppler echocardiography showed an enlarged left side of the heart, along with ventricular wall hypokinesis and compromised functioning of the same side of the heart. Abdominal color ultrasonography revealed that the patient\'s spleen was enlarged. Observations from cardiac magnetic resonance imaging showed that the left side of the heart was enlarged. Slight myocardical fibrosis was also observed. Electromyography was described as a symmetric sensorimotor demyelinating polyneuropathy. Further immunoelectrophoresis of the serum showed the presence of a monoclonal IGA λ M protein. The vascular endothelial growth factor levels were 622.56 pg/mL. Flow cytometric and immunohistochemical staining of the bone marrow detected no monoclonal plasma cells. Finally, the patient was diagnosed with POEMS syndrome associated with dilated cardiomyopathy. The chest-related discomfort and the shortness of breath resolved after the administration of lenalidomide and dexamethasone.
CONCLUSIONS: When patients with cardiomyopathy have systemic manifestations such as numb limbs and darkening skin, the POEMS syndrome is the most possible diagnosis.
METHODS: Herein, we report the case of a 59-year-old woman with POEMS syndrome that involved dilated cardiomyopathy. The patient presented to the hospital with complaints of shortness of breath and discomfort in the chest. The patient reported previous experiences of limb numbness. During hospitalization, the brain natriuretic peptide levels were 3504.0 pg/mL. Color doppler echocardiography showed an enlarged left side of the heart, along with ventricular wall hypokinesis and compromised functioning of the same side of the heart. Abdominal color ultrasonography revealed that the patient\'s spleen was enlarged. Observations from cardiac magnetic resonance imaging showed that the left side of the heart was enlarged. Slight myocardical fibrosis was also observed. Electromyography was described as a symmetric sensorimotor demyelinating polyneuropathy. Further immunoelectrophoresis of the serum showed the presence of a monoclonal IGA λ M protein. The vascular endothelial growth factor levels were 622.56 pg/mL. Flow cytometric and immunohistochemical staining of the bone marrow detected no monoclonal plasma cells. Finally, the patient was diagnosed with POEMS syndrome associated with dilated cardiomyopathy. The chest-related discomfort and the shortness of breath resolved after the administration of lenalidomide and dexamethasone.
CONCLUSIONS: When patients with cardiomyopathy have systemic manifestations such as numb limbs and darkening skin, the POEMS syndrome is the most possible diagnosis.
摘要：
背景：多发性神经病，器官肿大,内分泌病,M-蛋白,皮肤改变（POEMS）综合征是一种罕见的副肿瘤综合征，包括多个系统。POEMS综合征最常见的临床症状是进行性感觉运动性多发性神经病,器官增大,内分泌失调,皮肤变黑，一种单克隆浆细胞增殖性疾病,和淋巴结增生。器官肿大包括肝脾肿大和/或淋巴结肿大；心肌病的病例很少见。由于该综合征的非典型性，诊断通常会延迟，使患者可能严重残疾。因此,识别不典型症状可以改善POEMS综合征患者的预后和生活质量。
方法：这里，我们报道了一例59岁的POEMS综合征患者,该综合征涉及扩张型心肌病.患者出现在医院，抱怨呼吸急促和胸部不适。患者报告了先前的肢体麻木经历。住院期间,脑钠肽水平为3504.0pg/mL.彩色多普勒超声心动图显示心脏左侧扩大，伴随着心室壁运动功能减退和心脏同一侧的功能受损。腹部彩超显示患者脾脏肿大。心脏磁共振成像的观察显示心脏左侧扩大。还观察到轻微的心肌纤维化。肌电图被描述为对称的感觉运动脱髓鞘性多发性神经病。血清的进一步免疫电泳显示存在单克隆IGAλM蛋白。血管内皮生长因子水平为622.56pg/mL。流式细胞术和免疫组织化学染色的骨髓未检测到单克隆浆细胞。最后,患者被诊断为与扩张型心肌病相关的POEMS综合征.给予来那度胺和地塞米松后，胸部相关的不适和呼吸急促得以缓解。
结论：当心肌病患者出现四肢麻木、皮肤变黑等全身表现时，POEMS综合征是最可能的诊断。
方法：这里，我们报道了一例59岁的POEMS综合征患者,该综合征涉及扩张型心肌病.患者出现在医院，抱怨呼吸急促和胸部不适。患者报告了先前的肢体麻木经历。住院期间,脑钠肽水平为3504.0pg/mL.彩色多普勒超声心动图显示心脏左侧扩大，伴随着心室壁运动功能减退和心脏同一侧的功能受损。腹部彩超显示患者脾脏肿大。心脏磁共振成像的观察显示心脏左侧扩大。还观察到轻微的心肌纤维化。肌电图被描述为对称的感觉运动脱髓鞘性多发性神经病。血清的进一步免疫电泳显示存在单克隆IGAλM蛋白。血管内皮生长因子水平为622.56pg/mL。流式细胞术和免疫组织化学染色的骨髓未检测到单克隆浆细胞。最后,患者被诊断为与扩张型心肌病相关的POEMS综合征.给予来那度胺和地塞米松后，胸部相关的不适和呼吸急促得以缓解。
结论：当心肌病患者出现四肢麻木、皮肤变黑等全身表现时，POEMS综合征是最可能的诊断。
