浆细胞病伴POEMS综合征误诊为心脏疾患2例

例1女，56岁。因双下肢水肿伴疼痛、活动受限9个月余于2003年2月8日入院。患者于2002年5月突发左半肢体活动受限，后自行缓解。8月起出现双足趾麻木，双下肢凹陷性水肿，伴疼痛及活动受限，外院心脏彩超“左房增大，二、三尖瓣关闭不全”。肾功能正常。于2003年2月以“心衰?”入我院心内科治疗无效。腹部B超“肝脾肿大”。免疫球蛋白IgA11．1g／L。     

POEMS综合征一例

POEMS综合征是一种与浆细胞病有关的多系统损害疾病 ,可出现硬皮病样表现而就诊风湿科。我院近期收治 1例典型病例 ,报道如下。患者 :女性 ,4 6岁。因皮肤变黑、变硬、多毛 9个月 ,四肢麻木乏力、腹胀 4个月 ,于 2 0 0 2年 10月 18日收入院。患者 2 0 0 1年 1月因左结节性甲状腺肿伴囊性变行左甲状腺次全切除术 ,术后 1个月出现甲状腺功能低下 ,长期甲状腺素替代治疗。 2 0 0 1年 5月发现颈淋巴结肿大 ,病理示慢性炎症。 9个月前皮肤变黑、变硬 ,从四肢远端开始 ,渐及前臂、躯干及面部 ,伴体毛增多、声音稍变粗、性欲下降及闭经。 4个月前…     

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。     

以POEMS综合征为首发症状的骨髓瘤一例

POEMS综合征又名Crow Fukase综合征 ,为一种较为少见的临床综合征 ,发病机制不明 ,现认为是一种与浆细胞疾病有关的多系统病变。本院收治一例以POEMS综合征为首发症状 ,经过 5年才确诊的多发性骨髓瘤 ,现报道如下。患者女 ,5 8岁。于 1999年 4月无明显诱因出现双下肢无力 ,麻木     

POEMS综合征一例

男患,44岁,于2006年1月3日入院。该患者3年前开始全身皮肤逐渐发黑,形体消瘦。2003年11月感觉双脚胀、麻,走路时脚掌发厚,有凸凹不平感,近1年双腿无力,上楼费力。神经传导速度检查显示:神经传导速度减慢,诊断为“末梢神经炎”,用维生素B1、B12肌注,效果不佳。2005年1月查腹部彩超发现肝脾肿大,肝功能正常,5月出现双下肢浮肿,8月感冒后两次发热,体温38℃左右,抗菌素治疗后好转,10月又发热伴喘憋,查B超示:肝脾肿大,少量胸腔积液及腹水。经抗结核及激素治疗好转。近几年四肢汗毛逐渐增多;发病即伴性功能障碍;双乳房曾胀痛。查体:血压120/80mm…     

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综合征相关性肾病临床分析

目的回顾性分析POEMS综合征(五大病症首位字母缩写:多发性周围神经病,polyneuropathy,P;器官肿大organomegaly,O;内分泌障碍endocrinophthy,E;M-蛋白血症M-proteins,M;皮肤改变skinchanges,S。)相关性肾病的临床和病理特征。方法对上海长征医院1997-05~2003-08回顾性研究POEMS综合征的12例患者的临床、实验室及3例肾穿刺病理诊断结果。结果POEMS综合征相关性肾病发生率25%,最常见的表现为轻度至中度蛋白尿、水肿、镜下血尿;病理呈现膜增生性肾小球肾炎样病变,个别肾小球见λ轻链沉积。结论POEMS综合征相关性肾病临床症状相对轻微,主要表现为蛋白尿、血尿、肾功能损害。病理缺乏特异性,治疗主要采用激素治疗,预后差。     

POEMS综合征五例报告

POEMS综合征（又称Crow-Fukase综合征）是一种病因未明确的以多发性周围神经病变为主,伴有或不伴有多发性骨髓瘤的多系统损害征候群.其中主要以多发性周围神经病（Peripheral neuropathy,P）、脏器肿大（Organomegaly,O）、内分泌病变（Endocrinopathy,E）、M蛋白病变（Monoclonal gammophathy,M）、皮肤改变（Skin changes,S）为主要特征,还包括骨质代谢异常、大淋巴结增生症、视神经乳头水肿、腹水、胸腔积液,红细胞增多症、血小板增多症、乏力和杵状指[1].我们总结了2000～2006年期间在本院住院确诊的5例患者的临床特点,现报道如下：     

POEMS综合征23例临床分析

POEMS综合征又称Crow-Fukase综合征,是一种临床少见的多器官损害综合征,以进行性多发性周围神经病变(polyneuropathy,P)、器官肿大(organomegaly,O)、内分泌病变(endocrinopathy,E)、M蛋白增高(M protein,M)和皮肤改变(skin changes,S)为主要特征的一类疾病.临床表现复杂多样,易误诊、漏诊.本文总结了本院2000-2008年收治的23例患者临床资料,进行回顾性分析提高对本病的认识,现分析如下.     

POEMS综合征一例

ＰＯＥＭＳ综合征一例陈文军杨耀波戚沛霖患者男性，６８岁，因双下肢麻木、无力４个月于１９９２年１０月６日入院。患者于１９９２年６月起感左大腿麻木，继之右大腿麻木，渐感双腿无力，出现尿失禁，经肌肉注射维生素Ｂ１及Ｂ１２治疗无效。既往健康。查体：血压１６／...     

PRIMARY CEREBRAL LYMPHOMA: PRESENTATION OF EIGHT CASES AND REVIEW OF THE LITERATURE

Eight cases of primary cerebral lymphoma (PCL) are presented, being the largest Australian series of this rare condition reported to date. The clinical, radiological, anatomical and histological features were found to be comparable with those of patients reported in other series between 1954 to 1986. The precise treatment of PCL remains undetermined due to its rarity and apparent heterogeneity. Whole brain irradiation forms the basis of therapy although the role of prophylactic spinal irradiation, surgery and chemotherapy have not been defined. Response to corticosteroids and chemotherapy has been documented. Reported cases associated with the acquired immune deficiency syndrome (AIDS) are discussed. With an anticipated increase in the numbers of cases of AIDS, the incidence of related PCL will also increase. Large, multicentric trials are required to further delineate optimal treatment.     

THE NAIL-PATELLA SYNDROME--A REPORT OF TWO CASES AND A LITERATURE REVIEW

The nail–patella syndrome is a rare and curious conditionwhich affects both mesodermal and ectodermal structures. Wedescribe two case and also review the literature on the subject     

儿童肾上腺脑白质营养不良（附一例报告）

报告１例肾上腺脑白质营养不良的男童。该病是累及内分泌系统和神经系统的比较少见的Ｘ连锁遗传性过氧化酶体疾病，系由于饱和极长链脂肪酸在组织中储聚，引起神经系统脱髓鞘改变和肾上腺皮质功能减退。其临床表现多样，易误诊和漏诊，尚无特效治疗方法，预后不良     

二尖瓣腱索断裂(附10例报告及文献复习)

本文报告10例二尖瓣腱索断裂病例并复习近年国内文献。综合分析36例患者资料表明:二尖瓣腱索断裂以后叶常见,后、前叶累及比例为1.8:1。病因以自发性断裂常见,尤其是老年患者。青少年患者风湿性心脏炎是常见原因。感染性心内膜炎已不是二尖瓣腱索断裂的主要原因。     

ACUTE PSEUDOGOUT OF THE TEMPOROMANDIBULAR JOINT: A REPORT OF THREE CASES AND REVIEW OF THE LITERATURE

Three cases of acute pseudogout of the temporomandibular jointare described. The rarity of pseudogout in this joint may bepartly due to a failure to recognize this diagnosis as a causeof facial pain. KEY WORDS: Temporomandibular joint, Pseudogout, Chondrocalcinosis     

PANCREATIC TUBERCULOSIS AND ITS DIAGNOSIS BY ENDOSCOPIC ULTRASONOGRAPHY: REPORT OF TWO CASES AND REVIEW OF THE LITERATURE

Mass lesions in the head of the pancreas are generally malignant and it is difficult to diagnose benign lesions preoperatively. We describe two patients with pancreatic tuberculosis, who presented with abdominal pain, jaundice and a pancreatic head mass, mimicking cancer. The correct diagnosis could be made by endoscopic ultrasonography (EUS) and EUS‐guided fine‐needle aspiration (FNA) cytology in both patients, precluding the need for surgery. Both patients responded well to anti‐tuberculosis treatment. We conclude that EUS with guided FNA is a useful modality to diagnose pancreatic tuberculosis.     

POLYARTERITIS NODOSA IN HUMAN IMMUNODEFICIENCY VIRUS INFECTION: REPORT OF FOUR CASES AND REVIEW OF THE LITERATURE

Four patients with HIV infection were diagnosed as having panarteritisnodosa type vasculitis in muscle samples taken by biopsy. Fever,weight loss and neuromuscular symptoms were the major complaints.All but one were successfully treated with a low dose of prednisone.The other patient refused treatment, but her muscular complaintsdid not worsen during 9 months of follow-up. Previously reportedcases in the literature are reviewed KEY WORDS: AIDS, HIV, Necrotizing arteritis, Rheumatic manifestations, Vasculitis, Panarteritis nodosa, Prednisone, Neuromuscular symptoms, Muscle biopsy, Muscle atrophy     

HEPATITIS-ASSOCIATED APLASTIC ANEMIA AND ACUTE PARVOVIRUS B19 INFECTION: A REPORT OF TWO CASES AND A REVIEW OF THE LITERATURE

Hepatitis-associated aplastic anemia is rare in general, but occurs in up to 28% of patients receiving liver transplantation for fulminant non-A, non-B hepatitis. Cases are commonly young men with mild hepatitis but severe aplastic anemia. Although cases have been reported in association with hepatitis A, B, and C, most appear to be due to a non-A-B-C virus. We report two cases of acute hepatitis subsequently complicated by marrow hypoplasia in patients with acute parvovirus B19 infection. Hepatic manifestations of parvovirus B19 infection range from liver chemistry abnormalities to fulminant hepatic failure and aplastic anemia. Our cases demonstrate a less severe form of hepatitis-associated aplastic anemia, and together with other data, suggest that parvovirus B19 is at least one cause of hepatitis-associated aplastic anemia, and may be a heretofore underrecognized hepatotrophic virus.