一例腰椎管狭窄症患者术后发热并神经功能恶化引发的思考

本文报道一例腰椎管狭窄症患者术后症状一过性改善, 4周后再次加重, 并出现发热、双足下垂等症状, 经多科会诊后确诊为腰椎管狭窄症合并POEMS综合征, 予化疗及自体造血干细胞移植后症状改善。尽管POEMS综合征属罕见病, 同时合并腰椎管狭窄症极为罕见, 但遵循严格的先定位、再定性的神经疾病诊断流程, 重视临床工作中的疑点, 漏诊是可以避免的。     

Crow－Fukase综合征──尸检病例报告及文献复习

报告１例经尸检证实的Ｃｒｏｗ－Ｆｕｋａｓｅ综合征。患者女性，４９岁，同时患有多发性周围神经病，肝脾肿大，内分泌功能障碍及皮肤色素沉着，故该病也称之为ＰＯＥＭＳ综合征。结合文献，就其发病机制进行了简单讨论，并认为该病在临床并非少见，诊断率不高可能与对该病的认识不足有关。     

Successful weaning after plasma exchange for polyneuropathy related to POEMS syndrome

Respiratory failure due to polyneuropathy associated with POEMS syndrome (Polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) is unusual. In this report, we describe a 47‐year‐old male patient with polyneuropathy related to POEMS syndrome who suffered from hypercapnic respiratory failure due to progressive weakness of the respiratory muscles. His respiratory muscle strength significantly improved after plasma exchange and was extubated successfully. To our knowledge, this is the first case of POEMS syndrome with aggressive polyneuropathy that was successful treated by plasma exchange and our experience provides insight regarding the optimal management of such patients. J. Clin. Apheresis, 2009. © 2009 Wiley‐Liss, Inc.     

A case report: isolated a heavy chain monoclonal gammopathy in a patient with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin change syndrome

A 45-year-old South-Korean man presented with abdominal distension, progressive paresthesia and motor weakness of both lower extremities. Our case was identified as polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin change (POEMS) syndrome based on diagnostic criteria. Circulating M components of POEMS syndrome consist mainly of IgG or IgA-lambda and rarely IgM-lambda, IgG-kappa or isolated light chains. In this case, the M-band on serum protein electrophoresis and isolated IgA heavy chain on serum immunofixation electrophoresis were demonstrated, but there was no abnormal light chain. We suggest that this case may be associated with a pattern of abnormal secretion of monoclonal protein or a coincidence of a heavy chain disease in POEMS syndrome, even though the latter possibility may be very rare.     

POEMS综合征合并急性脑梗塞

目的　观察和探讨POEMS综合征与急性脑梗塞的内在相关性。方法　报道 1例确诊为POEMS综合征的男性病人 ,在病后半年突然出现左侧肢体完全偏瘫 ,通过对临床、血液有形成分、电生理及影像学进行研究。结果　血红细胞、血红蛋白、血小板计数及血细胞压积明显增高 ,肌电图显示广泛神经传导速度减慢 ,骨盆片可见骨硬化病灶 ,头颅MRI显示右侧大脑颞、额、顶、枕大面积脑梗塞。结论　POEMS综合征可以引起广泛的血管病变包括大血管、中小血管及毛细血管 ,成为导致血管闭塞的直接原因 ,而微血管病变可能在POEMS综合征的发病机制中起重要作用。     

Endocrinopathy in POEMS syndrome: the Mayo Clinic experience

OBJECTIVE: To determine the prevalence and characteristics of endocrinopathies at diagnosis of POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome. PATIENTS AND METHODS: From January 1, 1960, through June 30, 2006, we identified 170 patients with POEMS syndrome in the Mayo Clinic dysproteinemia database. We abstracted information about endocrine abnormalities from their medical records. RESULTS: Of the 170 patients with POEMS syndrome during the entire study period, the 64 patients seen after 2000 had more complete endocrine evaluations; of these 64 patients, 54 (84%) had a recognized endocrinopathy (38 men; median age, 50 years; interquartile range, 43-59 years). Hypogonadism was the most common endocrine abnormality; 26 (79%) of 33 men had subnormal total testosterone levels, and 10 men had gynecomastia. Among the 35 patients with measured prolactin levels, 7 men and 3 women had elevated levels. Hypothyroidism was noted in 17 men and 11 women. Abnormalities in glucose metabolism were present in 24 (48%) of 50 patients; 16 patients had impaired fasting glucose levels, and 8 were diagnosed as having diabetes. Adrenal insufficiency (defined by an abnormal response of cortisol to stimulation with standard high-dose [250 microg] synthetic adrenocorticotropic hormone) was noted in 6 of 9 patients tested. Fourteen (27%) of 51 patients tested had hypocalcemia. Twenty-nine (54%) of 54 patients had evidence of multiple endocrinopathies in the 4 major endocrine axes (gonadal, thyroid, glucose, and adrenal). CONCLUSION: The high prevalence of endocrinopathy in our study, to our knowledge the largest published series of POEMS cases, calls for a thorough endocrine investigation in patients presenting with this syndrome.     

Castleman病变异型POEMS综合征一例

本文报道1例以发热、胸腹水起病, 相继出现皮肤、内分泌系统及神经系统多系统受累的无M蛋白的Castleman病变异型POEMS综合征病例。通过对患者临床表现、实验室检查结果及治疗反应的分析, 加强临床医生对不典型POEMS综合征的认识。     

1例POEMS综合征的报告及护理初探

POEMS综合征是一组临床少见的多系统损害的症候群。本文对一例POEMS进行了报告并阐述了护理体会。作者通过心理护理、生活护理,成功的静脉穿刺,功能锻炼及卫生宣教、出院指导等一系列护理措施,使此病症状得到控制,病情得到缓解。     

POEMS综合征的免疫学检测结果分析

目的分析研究POEMS综合征的免疫特征，为临床诊断与治疗提供依据。方法回顾性地对27例POEMS综合征患者血清蛋白电泳（SPE）、免疫固定电泳（IFE）以及免疫球蛋白定量结果进行分析。结果27例POEMS综合征患者M蛋白检出率IFE明显高于SPE；M蛋白IgG5例，IgA16例，IgA出现几率明显高于IgG，且均是λ型；另外16例SPE检测出M带的血清蛋白电泳结果与健康对照组比较差异有统计学意义。结论M蛋白阴性不排除对POEMS综合征的诊断，对M蛋白阴性患者除进一步多途径寻找依据外，还应更多参考临床表现。     

POEMS综合征的临床与周围神经病理分析

目的：探讨POEMS综合征的临床和周围神经病理特点。方法：回顾性分析5例POEMS综合征的临床特征和4例周围神经活检结果。结果：5例患者临床上均有感觉运动性周围神经损害和肢端凹陷性水肿，腹水3例，胸腔积液2例，心包积液2例。肝脏肿大2例，脾脏肿大3例，淋巴结肿大2例。皮肤变黑5例，多毛症3例。4例腓肠神经活检中，3例神经束膜增厚，束间小血管增多，周围散在有单核细胞。4例均可见脱髓鞘改变和有髓纤维减少，3例可见到少数纤维轴索变性。单神经纤维剥离检查4例，均可见阶段性脱髓鞘改变和薄髓纤维。结论：POEMS综合征是一组以多发性神经病、脏器肿大、内分泌病变、M蛋白、皮肤损害等多系统损害的临床症候群。周围神经病理改变主要为薄髓纤维和脱髓鞘改变，晚期可见轴索变性。     

POEMS综合征22例临床分析

本研究探讨我国POEMS综合征的临床特点。回顾性分析中国人民解放军总医院22例POEMS综合征的临床特征。结果表明：患者平均年龄47．7岁，男：女=2．67；首发症状以无力最多；神经系统病变见于所有病例，主要表现为无力、麻木，肌电图神经源性损害，脑脊液常有压力和蛋白升高。脑脊液蛋白电泳可见单克隆带阳性；肝脾、淋巴结肿大常见；内分泌病变占95．5％，以性腺功能减退最为常见；M蛋白检查中以免疫固定电泳阳性率最高为92．9％，免疫球蛋白类型中IgA多于IgG，轻链以入型多见，但小部分患者出现K型轻链。皮肤改变为色素沉着、多毛、多汗。结论：本组病例临床特征基本上与国内外报道的相似，但与国外报道病例不完全相同，有一定差异。     

沙利度胺联合VAD方案治疗骨硬化性骨髓瘤(POEMS综合征)临床观察

本研究旨在探讨骨硬化性骨髓瘤(POEMS综合征)的临床特征,评价沙利度胺联合VAD方案治疗POEMS综合征的疗效。回顾性分析2006年6月至2011年7月我院收治的27例POEMS综合征患者的临床表现、实验室检查、治疗及预后情况。结果表明:27例POEMS患者具有多发性周围神经病变(27/27)、肝脾淋巴结肿大(15/27)、内分泌改变(24/27)、皮肤病变(22/27)等典型临床表现及血清M蛋白阳性(23/27);此外,还有周围性水肿、浆膜腔积液、视盘水肿、骨硬化病变等。用沙利度胺联合VAD方案治疗POEMS综合征缓解率分别为脏器肿大60%,水肿58.3%,皮肤病变41%,内分泌病变45.8%;治疗后IgG/λ型、IgA/λ型患者血清M蛋白水平均显著下降,与治疗前相比差异有统计学意义(P<0.05);神经系统ODSS值与治疗前相比差异有统计学意义(P<0.01)。结论:POEMS综合征临床特征复杂易误诊,血清M蛋白阴性的疑诊患者应积极寻找单克隆浆细胞增生的证据;用VAD方案联合沙利度胺治疗POEMS综合征疗效明显,副作用少,患者耐受性好,且安全性较高,在临床可作为首选的治疗途径。     

以腹水为突出表现的M蛋白阴性的POEMS综合征变异型一例

POEMS综合征是与浆细胞疾病相关的一种临床少见病。经典五联征包括：多发性周围神经病、器官肿大、内分泌障碍、M蛋白血症和皮肤病变。由于其罕见性、多系统受累及临床高度异质性,漏诊率和误诊率均较高。现报道1例以腹水为突出表现的M蛋白阴性的POEMS综合征变异型。     

多发性神经病变、器官肿大、内分泌异常、M蛋白、皮肤病变综合征99Tcm-MDP全身骨显像分析

目的 探讨多发性神经病变、器官肿大、内分泌异常、M蛋白、皮肤病变（POEMS）综合征骨显像表现及临床价值。方法 回顾性分析8例POEMS综合征患者的临床、影像学资料,与CT表现进行比较,总结POEMS综合征的骨显像特点。结果 8例患者中多发性骨髓瘤3例,浆细胞增生3例,浆细胞瘤2例。6例骨显像阳性,其中2例为溶骨性破坏灶,4例为硬化性病变。5例可见四肢关节对称性放射性浓聚,其中1例治疗后好转。结论 POEMS综合征溶骨性病变及较大硬化性病灶呈放射性浓聚,伴四肢关节对称性浓聚;骨显像有助于其早期诊断及病情评价。     

POEMS综合征425例临床分析

目的总结425例POEMS综合征的临床特点及诊治.方法荟萃总结分析POEMS综合征临床表现的特点并与国外文献相比较.结果 POEMS综合征多见于中年男性,发病年龄11～74岁,平均43岁;临床表现以多发性周围神经病、脏器肿大、内分泌病、M-蛋白和皮肤改变为特征,常与多发性骨髓瘤并存;死因多见于严重感染、呼吸衰竭或心力衰竭等,发病机制不详;激素疗法短期疗效尚可.结论国内POEMS综合征患者周围神经损害、皮肤改变、肢端水肿等较常见,其次为肝脾肿大、阳痿、男性女性化乳房、闭经、胸腹水等.     

Sonographic findings in a case of polyneuropathy associated with POEMS syndrome

Polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes (POEMS) syndrome is a rare multi-system disease. We describe the ultrasonography (US) and color Doppler ultrasonography (CDUS) findings of peripheral neuropathy in a patient with POEMS syndrome. In US examination, peripheral nerves were found to be diffusely thickned in both upper limbs. CDUS imaging showed arterial blood flow with low systolic peaks on the nerves.     

Macrophage activation syndrome as an initial presentation of systemic lupus erythematosus

In a recent article on World J Clin Cases 2019; 7: 3859-3865, Sun et al reported a case of 36-year-old female with macrophage activity syndrome as an onset of systemic lupus erythematosus. Although this is a very interesting case, some concerns still need to be addressed. First, the patient had an extremely elevated serum ferritin but a normal C-reactive protein level, which was unparallel with the inflammatory condition before she received any treatments. Second, the diagnosis of systemic lupus erythematosus seemed to be insufficient according to the patient’s medical information presented, most of which were not specific to lupus but could be explained by macrophage activity syndrome. Hence, more medical information on the patient should be provided, and a profound discussion needs to be addressed.     

Cutaneous Mycobacterium chelonae infection following autologous peripheral blood stem cell transplantation for POEMS syndrome

Although hematopoietic stem cell transplantation (HSCT) may increase the curability of refractory hematologic diseases, it requires complication management due to a long-term immunocompromised state. We experienced a case who received an autologous peripheral blood stem cell transplantation (Auto-PBSCT) for POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) and developed cutaneous Mycobacterium chelonae infection. It is clear that attention needs to be paid to prevent bacterial, fungal and viral infection after HSCT. It is also important to keep in mind that tuberculous and nontuberculous mycobacteria (NTM), in rare cases, lead to lethal complications.     

Eisenmenger syndrome: A clinical review

Eisenmenger syndrome is a medical condition, characterized by elevated pulmonary vascular resistance and right-to-left shunting of blood through a systemic-to-pulmonary circulation connection. Patients with Eisenmenger syndrome are not very prevalent in cardiovascular nurses' practice. However, nurses need to have some basic knowledge about the syndrome, in case they have to care for such a patient in their clinical practice. In this article, we describe the epidemiology, outcome, pathophysiology, clinical presentation, medical management and counseling issues of this condition.     

Acoustic radiation force impulse elastography and contrast-enhanced sonography of sinusoidal obstructive syndrome (Veno-occlusive Disease): preliminary results

We report quantitative liver acoustic radiation force impulse (ARFI) elastographic findings in 2 cases of sinusoidal obstructive syndrome and liver contrast-enhanced sonographic features in one of these cases. To our knowledge, findings in this condition from these techniques have not been reported previously. Acoustic radiation force impulse elastography showed median high shear wave velocities (case 1, 2.75 m/s; case 2, 2.58 m/s) that normalized after specific treatment for sinusoidal obstructive syndrome; therefore, ARFI elastography provided quantitative information that helped diagnose this condition as well as monitor the response to treatment. Contrast-enhanced sonographic findings in one of the cases showed patchy liver enhancement that correlated with the high-velocity patchy distribution on ARFI elastography in that case and enhanced multidetector row computed tomographic findings in the other case. This contrast-enhanced sonographic pattern progressively normalized during follow-up after specific treatment. The elastographic features in both cases and contrast-enhanced sonographic features in one of them contributed to early diagnosis and follow-up of sinusoidal obstructive syndrome in both patients. Further prospective studies are necessary to define the role of ARFI elastography and contrast-enhanced sonography in the early diagnosis and clinical follow-up of this condition.