skin changes syndrome

皮肤改变综合征
  • 文章类型: Case Reports
    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。
    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.
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  • 文章类型: Case Reports
    多发性神经病,器官肿大,内分泌疾病,M-蛋白,皮肤变化(POEMS)综合征是一种罕见的疾病,具有多种表现和一系列症状。我们介绍了一名62岁的女性,她因急性呼吸困难而向急诊科就诊。胸部X光显示肋骨和胸椎硬化病变。计算机断层扫描(CT)和正电子发射断层扫描(PET)扫描的进一步成像研究暗示了良性过程。通过支持性管理看到了改善。几个月后,患者出现神经系统症状,运动耐量降低。肌电图诊断为混合性脱髓鞘和轴索多发性神经病。进一步的骨髓活检和免疫化学检测显示λ和κ浆细胞疾病,血管内皮生长因子(VEGF)升高。患者被诊断为POEMS并开始化疗。POEMS综合征由于其罕见且临床表现多样,通常被错过。VEGF在诊断中起着至关重要的作用。管理需要多学科方法。
    Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin changes (POEMS) syndrome is a rare disorder with multiple presentations and a constellation of symptoms. We present a 62 year-old female who presented to the Emergency Department for acute dyspnea. Chest Xray showed sclerotic lesions in the ribs and thoracic spine. Further imaging studies with computed tomography (CT) and positron emission tomography (PET) scans were suggestive of a benign process. Improvement was seen with supportive management. A few months later, patient developed neurological symptoms with reduced exercise tolerance. Mixed demyelinating and axonal polyneuropathy was diagnosed by electromyography. Further work up with bone marrow biopsy and immunochemistry testing revealed lambda and kappa plasma cell disorder, with elevated vascular endothelial growth factor (VEGF). Patient was diagnosed with POEMS and initiated on chemotherapy. POEMS syndrome is commonly missed due to its rarity and varied clinical presentations. VEGF plays a crucial role in the diagnosis. Management requires a multidisciplinary approach.
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  • 文章类型: Case Reports
    我们报告了一名年轻男性逐渐发展为亚急性脱髓鞘性多神经根神经病的病例。常规检查和广泛的生化检查显示脱髓鞘性神经病,电泳中的M带。患者转诊为全身18F-氟代脱氧葡萄糖-正电子发射断层扫描/计算机断层扫描以寻找骨骼和骨髓病变。患者被发现患有肋骨浆细胞瘤。这种情况很少见,因为与网站的联系很少,年龄,和症状。
    We report the case of a young male who gradually developed a subacute demyelinating polyradiculoneuropathy. Routine examination and extensive biochemical investigations revealed demyelinating neuropathy, M-Band in electrophoresis. The patient referred for the whole-body 18F-fluorodeoxyglucose-positron emission tomography/computed tomography to look for skeletal and bone marrow lesions. The patient was found to have rib plasmacytoma. The case is rare because of infrequent association with the site, age, and symptoms.
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  • 文章类型: Case Reports
    Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes (POEMS) syndrome is a systemic condition related to plasma cell dyscrasia. Increased vascular permeability is responsible for some of the hallmarks of this disorder that may include renal microangiopathy. Several lines of evidence suggest that vascular endothelial growth factor (VEGF) is central to the pathogenesis of POEMS syndrome. Thus, specifically targeting VEGF over-expression seems to be a promising treatment. Anti-VEGF therapies are yielding conflicting results. We report on a patient with POEMS syndrome treated with bevacizumab, an anti-VEGF monoclonal antibody. Sequential monitoring of serum VEGF showed sustained normalization of serum VEGF levels, without any beneficial effect on the patient\'s condition. Indications of this treatment should be chosen carefully.
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