POEMS综合征10例临床分析

目的 :分析POEMS综合征的临床特征 ,以提高对本病的诊治水平。方法 :采用回顾性方法对 10例POEMS综合征的临床资料进行分析。结果 :10例患者中 ,主要临床表现为多发性神经病变 (10 0 % )、肝、脾肿大 (分别为 4 0 %、90 % )、内分泌病变 (10 0 % )、M蛋白血症 (10 0 % )、皮肤病变 (80 % )、水肿 (80 % ) ,10例患者中 8例经美法仑、强的松治疗后症状缓解 ,2例用地塞米松疗效不佳。结论 :POEMS综合征临床表现复杂多样 ,常易误诊 ,应予以重视     

POEMS综合征5例临床分析

背景：POEMS综合征是一组以多发性周围神经病为突出表现的临床症候群。该病少见且临床表现复杂、无特异性，极易漏诊或误诊。目的：总结POEMS综合征的临床特点，提高对该病的认识。方法：回顾性分析上海仁济医院2001年5月-2007年5月5例确诊的POEMS综合征患者的临床表现、实验室检查结果、治疗方案和预后情况。结果：5例POEMS综合征患者的主要临床表现发生率分别为周围神经病变、内分泌异常和皮肤改变100％．脏器肿大80％，M蛋白阳性60％。经激素或免疫抑制剂治疗，出院时4例患者好转，1例恶化，但至随访结束时仅1例维持好转。结论：POEMS综合征临床表现复杂。随着其诊断和治疗的进展，患者预后将明显改善。     

POEMS综合征：1例报告并文献复习

POEMS综合征是一种与浆细胞有关的多系统病变,临床上以进行性多发性周围神经病变（P）、脏器（肝脾）肿大（O）、内分泌紊乱（E）、M蛋白增高（M）和皮肤改变（S）为特征,常伴有全身凹陷性水肿、胸腹水、杵状指（趾）和心力衰竭等症状。我们报道1例男性38岁患者,因“腹胀、双下肢水肿2年,四肢远端麻木2月”入院,经临床各种检查被诊断为该病。我们复习了有关POEMS综合征的临床特征和诊治进展。     

老年人POEMS综合征1例

POEMS综合征是一种较少见的多系统损害症候群，常见于中青年因其常见临床表现为：多发性神经病变（polyneuropathy）、器官肿大（organomegaly）、内分泌病（endocrinopathy）、M蛋白和皮肤改变（skinchanges）而命名，亦称Crow Fukase综合征。POEMS综合征累及多系统，症状多样化，临床少见。解放军第254医院收治1例，现报道如下。[第一段]     

POEMS综合征误诊临床分析

目的探讨POEMS综合征的发病特点及临床表现。方法 2017年1月至2019年8月就诊于北京佑安医院的4例患者,根据就诊原因、临床表现,检查结果,对上述患者进行相关误诊分析。结果 1例患者以周围神经为首发症状,其余3例因腹腔积液、肝脾肿大就诊,免疫球蛋白A单株峰y轻链型3例,免疫球蛋白G单株峰y轻链型1例,4例VEGF均大于800 pg/mL,病理无肝纤维化表现。结论 POEMS综合征是多系统的病变,其发病率低,误诊率高。肝、脾肿大合并腹腔积液可能是部分POEMS综合征患者的首发症状。     

POEMS综合征合并糖尿病三例并文献复习

汇报POEMS综合征合并糖尿病3例。3例患者均以周围神经病变为首发症状,同时患有糖尿病,初步诊断考虑糖尿病周围神经病变,但经过相关检查最终确诊为POEMS综合征。POEMS综合征是发病率较低的一种浆细胞克隆性病变,大多数患者以多发性周围神经病变首发起病。本文归纳了POEMS综合征多发性周围神经病变与糖尿病周围神经病变的鉴别诊断要点。     

POEMS综合征相关肾损害临床及病理特征

目的:分析POEMS综合征的临床表现,肾脏损害的临床和病理特征,以提高对此病认识. 方法:回顾性分析POEMS综合征患者临床表现、实验室检查特点及肾活检病理资料. 结果:12例患者中男性9例,女性3例,诊断时平均年龄44.5±9.2岁.所有患者早期均误诊,发病至确诊时间为18.4±13.2月.临床特征:(1)多发性周围神经病变:四肢麻木、肌无力12例(100％),肌电图均示神经源性改变.(2)脏器肿大:肝肿大4例(33.3％)、脾肿大11例(91.7％)、淋巴结肿大4例(33.3％)、肾脏体积增大6例(50％).(3)内分泌病变:男性性功能障碍7例(77.7％)、女性闭经2例(66.7％),甲状腺功能减低10例(83.3％),糖尿病6例(50％).(4)单克隆浆细胞增生,血清M蛋白阳性率9例(75％).(5)皮肤色素沉着:所有患者均有皮肤色素沉着.(6)肾脏损害:水肿11例(91.7％);少量蛋白尿8例(66.7％);镜下血尿1例(8.3％);血清肌酐升高6例(50％);尿酸升高11例(91.7％).4例患者行肾活检,病理特点:(1)肾小球系膜病变明显(系膜区增宽、系膜细胞及基质增生、系膜溶解);(2)肾小球内皮细胞病变突出;(3)未见免疫复合物沉积. 结论:POEMS综合征临床累及多系统,症状复杂,容易漏诊、误诊.肾脏损害并非少见,少量蛋白尿为主,可伴肾功能不全,病理活检组织学改变以肾小球内皮细胞和系膜病变突出.     

蓬塞综合征11例误诊分析

蓬塞综合征又称结核性风湿病,是由结核杆菌引起的变态反应疾病,以发热、关节疼痛、皮肤损害及其特征。由于临床医生对本病认识不足,且不少慢性疾病与其表现类似,故极易误诊。1994年以来,我院收治本病患者13例,其中误诊11例,误诊率达84.6%。为提高对本病的认识,减少误诊,现将误诊的11例分析如下。     

POEMS综合征误诊病例分析

目的:分析被误诊的POEMS综合征的临床特点及误诊原因,以提高临床正确诊断率.方法:检索CBM-DISC、CNKI、万方、维普和PubMed相关数据库中被误诊的POEMS综合征个案报道及病例分析的资料,对误诊病例的症状、体征、实验室检查和误诊原因透行分类统计.结果:79例被误诊的病例中78.3％的病例以多发性神经病与吉兰巴雷综合征为首发症状,100.0％有多发性神经病表现,57.O％有肝肿大,70.0％有脾脏肿大,90.0％有皮肤色素沉着,81.0％有外周水肿,54.1％检测出M蛋白血症,88.6％脑脊液呈蛋白-细胞分离,骨髓象50.0％存在浆细胞异常增生,误诊疾病达23种,以误诊多发性神经病(34.2％)和吉兰巴雷综合征(11.4％)最多见.结论:对不明原因的周围神经病,需加强对M-蛋白血症和内分泌紊乱的检测,以提高对POEMS综合征的诊断.     

POEMS综合征32例临床分析

目的加深临床医师对POEMS综合征的认识,比较国内外POEMS综合征患者的临床特征。方法回顾性分析北京协和医院32例POEMS综合征患者的临床特征,并与国内外大宗病例报告进行比较。结果32例患者,平均年龄45、8岁,男女比例2．2：1。临床特征：（1）多发性周围神经病（P）：麻木（84．4％）、感觉异常（87．5％）、肌无力（90．6％）等。肌电图92．9％为神经源性改变,神经活检75．0％为轴索变性。（2）脏器肿大（O）：肝肿大71．9％、脾肿大87．5％、淋巴结肿大62．5％。9例淋巴结活检中5例为Castleman病。（3）内分泌病变（E）：男性勃起功能障碍72．7％、女性月经改变60．0％。（4）单克隆浆细胞增生（M）：血清M蛋白阳性率72．0％（18／25）。（5）皮肤色素沉着（S）：93．8％的患者有皮肤色素沉着。与其他报道相比,临床特征基本相似,但视乳头水肿、脾肿大、多汗等方面有一定差异;M蛋白的发生率与国外报道无差异,显著高于国内既往报道。结论本组POEMS综合征临床特征与国外基本相似,血清M蛋白的发生率并不低于国外。免疫电泳以及骨髓活检轻链组化染色可能有助于提高“M”的检测敏感性。     

Pulmonary hypertension in POEMS syndrome

POEMS syndrome is a rare clonal plasma cell disease. Patients with POEMS syndrome are at risk of developing pulmonary hypertension, but the data on its incidence and impact on outcome are limited. We reviewed records of 154 POEMS syndrome patients with complete duplex echocardiography data for estimation of pulmonary artery systolic pressure (sPAP) at the time of diagnosis. Forty-two (27%) of 154 patients with pulmonary hypertension (estimated sPAP ≥50mmHg) were identified. Median age was 46 years (range 31–71 years). Patients with pulmonary hypertension were more likely to have peripheral edema (P=0.04), ascites (P=0.02), pleural effusion (P=0.005), and have longer time from onset to diagnosis (P=0.004) when compared with those without pulmonary hypertension. Restrictive abnormalities and decreased diffusion capacity of carbon monoxide were observed in 83% and 96% patients with pulmonary hypertension, compared with 50% and 72% in patients without pulmonary hypertension, respectively. Reversibility of pulmonary hypertension was observed after treatment of POEMS syndrome. After median follow of 32 months, survival of patients with pulmonary hypertension was worse than those without (median overall survival 54 months vs. median not reached, P=0.021). In conclusion, pulmonary hypertension is a common feature of POEMS syndrome, and is associated with signs of extravascular volume overload. Although active treatment of POEMS syndrome can reverse pulmonary hypertension, survival of these patients is worse than those without pulmonary hypertension.     

Efficacy of lenalidomide in POEMS syndrome: A retrospective study of 20 patients

POEMS syndrome is a rare disorder characterized by polyneuropathy, monoclonal gammopathy, multiorgan involvement, and elevated vascular endothelial growth factor levels. Localized bone lesions require irradiation, whereas young patients with disseminated disease receive intensive treatment with stem cell support. Treatment of older and non responding patients is not yet standardized. We report the use of a combination of lenalidomide and dexamethasone in 20 patients with POEMS syndrome. Four patients were newly diagnosed, and 16 had relapsed or progressed after treatment. All but one of the patients responded: clinical improvements were noted in neuropathies (16/20) organomegaly (13/13), peripheral edema (14/15), and pulmonary hypertension (5/5). At least a very good partial response was noted in 68% of patients, with partial responses in 26%. Serum VEGF levels fell markedly in all 17 patients with available values. Twelve patients had 18‐FDG‐PET/CT at diagnosis (11 with positive findings), and nine patients during follow‐up. The number of lesions fell markedly in five cases and remained stable in two cases, while two patients became negative. During a median follow‐up of 22 months, four patients relapsed. Toxicity, predominantly hematological, was mild and manageable. Lenalidomide thus appears to be effective in POEMS syndrome, inducing high rate of clinical and biological responses. Am. J. Hematol. 88:207–212, 2013. © 2012 Wiley Periodicals, Inc.     

Combination of melphalan and dexamethasone for patients with newly diagnosed POEMS syndrome

POEMS syndrome is a rare clonal plasma cell disorder without standard treatment. Based on the efficacy and low toxicity of a combination of melphalan and dexamethasone (MDex) for light chain amyloidosis, we conducted a prospective study of MDex treatment for patients with newly diagnosed POEMS syndrome. Thirty-one patients (19 men) were enrolled and the median age at the time of diagnosis was 44 years (range, 32-68 years). All patients received 12 cycles of MDex treatment. Twenty-five patients (80.6%) achieved hematologic response including 12 (38.7%) complete remission and 13 (41.9%) partial remission. Of all 31 patients, the neurologic response rate was 100%, assessed by overall neuropathy limitation scale (ONLS). The initial neurologic response was observed in 24 patients (77.4%) at 3 months after treatment and the median time to maximal neurologic response was 12 months (range, 3-15 months). Moreover, MDex substantially improved the level of serum vascular endothelial growth factor and relieved organomegaly, extravascular volume overload, and pulmonary hypertension. Only 6 patients (19.3%) suffered from grade 3 adverse events during treatment. All patients are alive and free of neurologic relapse after the median follow-up time of 21 months. Therefore, MDex is an effective and well-tolerated treatment option for patients with newly diagnosed POEMS syndrome.     

136例POEMS综合征患者内分泌代谢异常的临床特点回顾性分析

目的 对POEMS综合征患者人群内分泌代谢异常的情况和临床特点进行分析和总结.方法 对2000年1月至2020年1月中国人民解放军总医院第一医学中心临床确诊的136例POEMS综合征患者的病例资料进行系统回顾性分析.结果 93.38％(127/136)的患者存在内分泌代谢异常,病变累及性腺、甲状腺、肾上腺、胰岛等多个内...     

POEMS syndrome treated with melphalan high-dose therapy and autologous blood stem cell transplantation: a single-institution experience

The acronym POEMS syndrome stands for a rare multi-system disorder, comprised of polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes. Here, we present a single-center report of a series of five POEMS patients treated with melphalan high-dose therapy (HDT) with subsequent autologous blood stem cell transplantation (ABSCT). After a median follow-up of 52 months from time of diagnosis (range, 15-192) and a median follow-up of 18 months after ABSCT (range, 11-120), all patients were alive. Overall, no severe transplantation-associated complications such as engraftment syndrome or peri- or post-transplant death were noted. In two cases, HDT followed by ABSCT resulted in a complete hematologic response; in the additional three cases, partial responses (PR) were achieved including one very good hematologic PR. Only one patient with initial PR developed progressive disease nearly 2.5 years after transplantation. Consequently, a second HDT with ABSCT was successfully applied resulting in clinical improvement and hematologic PR. In line with previous single-center reports, melphalan HDT followed by ABSCT proved to be a first-line treatment option with tolerable side effects in severely affected POEMS patients with progressing symptoms.     

POEMS syndrome: definitions and long-term outcome

The POEMS syndrome (coined to refer to polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) remains poorly understood. Ambiguity exists over the features necessary to establish the diagnosis, treatment efficacy, and prognosis. We identified 99 patients with POEMS syndrome. Minimal criteria were a sensorimotor peripheral neuropathy and evidence of a monoclonal plasmaproliferative disorder. To distinguish POEMS from neuropathy associated with monoclonal gammopathy of undetermined significance, additional criteria were included: a bone lesion, Castleman disease, organomegaly (or lymphadenopathy), endocrinopathy, edema (peripheral edema, ascites, or effusions), and skin changes. The median age at presentation was 51 years; 63% were men. Median survival was 165 months. With the exception of fingernail clubbing (P =.03) and extravascular volume overload (P =.04), no presenting feature, including the number of presenting features, was predictive of survival. Response to therapy (P <.001) was predictive of survival. Pulmonary hypertension, renal failure, thrombotic events, and congestive heart failure were observed and appear to be part of the syndrome. In 18 patients (18%), new disease manifestations developed over time. More than 50% of patients had a response to radiation, and 22% to 50% had responses to prednisone and a combination of melphalan and prednisone, respectively. We conclude that the median survival of patients with POEMS syndrome is 165 months, independent of the number of syndrome features, bone lesions, or plasma cells at diagnosis. Additional features of the syndrome often develop, but the complications of classic multiple myeloma rarely develop.     

Clinical characteristics and long-term outcome of patients with POEMS syndrome in China

POEMS syndrome is a rare plasma cell dyscrasia characterized by polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes. This study reviewed the clinical characteristics and long-term outcome of 99 consecutive Chinese patients with newly diagnosed POEMS syndrome in a single institute. The median age of 99 patients was 45 years, and the ratio of men/women was 1.4. The median time from onset of symptoms to diagnosis was 18 months. The typical five features of peripheral neuropathy, organomegaly, endocrinopathy, M protein, and skin change remained to be essential for patients with POEMS syndrome in China. The unusual features like pulmonary hypertension (36%) and renal impairment (37%) were not uncommon in China. Eighty-three percent patients were alive after follow-up time of 25 months, and 10% patients had survived more than 60 months. Melphalan-based therapy (OR, 0.076; 95% CI, 0.02–0.285) and normal renal function (OR, 0.246; 95% CI, 0.076–0.802) were independent prognostic factors for the survival of patients with POEMS syndrome. In conclusion, POEMS syndrome in Chinese patients was a multi-systemic disease with clinical features similar to non-Chinese ones. Active therapy can effectively improve the prognosis of patients with POEMS syndrome.     

A case of atypical POEMS syndrome without polyneuropathy

POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin changes) syndrome is a rare hematological disease associated with overproduction of pro-inflammatory cytokines. Under the current nomenclature and diagnostic criteria for POEMS syndrome, the presence of characteristic polyneuropathy is required for diagnosis. We report a 43-year-old Japanese woman with organomegaly, endocrinopathy, M-protein, skin lesions, as well as typical renal lesions and sclerotic bone lesions. Of note, neurological examinations and peripheral nerve conduction tests were normal in this patient. In view of the overwhelming number of otherwise characteristic signs and symptoms, we made a provisional diagnosis of 'atypical POEMS syndrome without polyneuropathy'. If further similar cases are reported in the future, reconsideration of the nomenclature and/or diagnostic criteria for POEMS syndrome may be required.