POEMS综合征合并肺动脉高压—临床分析及文献复习

发布时间：2018-05-22 20:41

本文选题：POEMS综合征 + 肺动脉高压　；参考：《浙江大学》2017年硕士论文

【摘要】：背景与目的:肺动脉高压是指肺动脉压力超过一定界限的一种血流动力学异常状态,本身并非一种独立的疾病,它即可来源于肺血管本身的病变,也可继发于其他系统疾病。POEMS综合征又称为Crow-Fukase综合征,是一种可累及皮肤、淋巴结、肝、脾、肺、肾、内分泌系统、神经系统及血液系统等多器官、多系统的浆细胞异常增生性疾病。患者的临床症状往往缺乏特异性,可并发包括肺动脉高压在内的多种并发症。POEMS综合征合并肺动脉高压的患者的临床表现多样,起病缓急不一,对临床医生诊断本病带来许多困难,临床误诊、漏诊率极高。本文通过2例POEMS综合征合并肺动脉高压患者的临床报道,并收集同时期我国文献报道的病例,分析总结该类疾病的临床特点、辅助检查、治疗及转归等特点,以提高对该疾病的认识。方法:采用病例检索系统,收集2013年1月至2017年1月在浙江大学医学院附属邵逸夫医院住院诊治的POEMS综合征合并肺动脉高压病例。并以"POEMS综合征"和"肺动脉高压"为关键词检索数据库在相同时期报道的我国病例,收集其临床特点、辅助检查、治疗及转归等资料,结合文献进行分析。结果:病例共计6例,男性1例,女性5例,年龄分布为35-74岁,平均年龄为52.33岁。6例患者的主要临床症状为气急、外周水肿、四肢麻木、皮肤改变,乏力等,部分患者有纳差,口干,咳嗽等症状。实验室检查中多数患者存在血沉增快、血氧饱和度降低、甲状腺功能减退等情况,仅有少数出现血常规异常。影像学检查提示最为多见的阳性结果为胸腔积液、心包积液、多发淋巴结肿大、脾大,部分患者存在肝大、腹水等表现。所有患者均存在M蛋白血症和周围神经病变,2例经淋巴结活检病理结果提示存在Castleman病,1例患者并发了慢性肾衰竭,1例患者还并发了男性乳房发育。6例患者从起病到明确诊断的时间平均为5年,最短2个月,最长达16年。6例患者在治疗后临床症状都得到明显缓解,出院后2例患者失访,1例随访2年后死亡,3例随访至今。结论:POEMS综合征合并肺动脉高压患者临床误诊率极高,其临床表现、病程进展、辅助检查结果具有多样性,常出现漏诊与误诊。相较于通常的肺动脉高压患者,POEMS综合征合并肺动脉高压患者还常伴有皮肤改变、脏器肿大、周围神经病、M蛋白血症、内分泌异常、血管内皮生长因子(vascular endothelial growth factor,VEGF)升高等表现。在详细询问病史,充分关注患者的体征,对于原发病难以解释的症状加以关注后,完善相关检查可明确诊断本病。目前临床上常采用糖皮质激素联合免疫抑制剂治疗本病,可获得较明显的治疗效果。
[Abstract]:Background & objective: pulmonary hypertension refers to an abnormal state of hemodynamics in which the pulmonary artery pressure exceeds a certain limit. It is not an independent disease in itself. It can be derived from the pathological changes of the pulmonary vessels themselves. Also secondary to other systemic diseases. Poems syndrome, also known as Crow-Fukase syndrome, is a multi-organ involving the skin, lymph nodes, liver, spleen, lung, kidney, endocrine system, nervous system and blood system. Multisystem plasmacyte abnormal proliferative disease. The clinical symptoms of patients are often lack of specificity, can be complicated with various complications, including pulmonary hypertension. It is difficult for clinicians to diagnose this disease, and the rate of missed diagnosis is very high. Two cases of POEMS syndrome complicated with pulmonary hypertension were reported, and the clinical characteristics, auxiliary examination, treatment and outcome of the disease were analyzed and summarized by collecting the cases reported in the literature of the same period in China. To raise awareness of the disease. Methods: cases of POEMS syndrome complicated with pulmonary hypertension were collected from January 2013 to January 2017 by case retrieval system in run Shaw Hospital affiliated to Zhejiang University Medical College. POEMS syndrome and pulmonary hypertension were used as key words to search the database of Chinese cases reported in the same period. The data of clinical characteristics, auxiliary examination, treatment and outcome were collected and analyzed in combination with literature. Results: there were 6 cases (1 male and 5 female). The age distribution was 35-74 years old. The main clinical symptoms of 6 cases with mean age of 52.33 years were acute qi, peripheral edema, limb numbness, skin change and fatigue. Some of the patients had anorexia. Dry mouth, cough, etc In laboratory examination, most patients had rapid ESR, decreased blood oxygen saturation and hypothyroidism, and only a few patients had abnormal blood routine. Imaging examination showed that the most common positive results were pleural effusion pericardial effusion multiple lymphadenomegaly splenomegaly and some patients with hepatomegaly and ascites. All patients had M proteemia and peripheral neuropathy. The pathological results of lymph node biopsy showed that 1 patient with Castleman's disease complicated with chronic renal failure and 1 patient with male breast development. The average time to be diagnosed is five years. The shortest 2 months and the longest 16 years 6 cases were obviously relieved after the treatment. 2 cases lost the visit after discharge and 1 case died after 2 years follow-up. 3 cases have been followed up so far. Conclusion the misdiagnosis rate is very high in the patients with points syndrome complicated with pulmonary hypertension. The clinical manifestations, progression of the disease course and the results of auxiliary examination are diverse, and missed diagnosis and misdiagnosis are often found. Compared with the patients with common pulmonary hypertension, patients with poems syndrome complicated with pulmonary hypertension were often accompanied by skin changes, organ enlargement, peripheral neuropathy with M proteemia, endocrine abnormalities, and the increase of vascular endothelial growth factor (VEGF) and vascular endothelial growth factor (VEGF). After inquiring the history of the disease in detail, paying full attention to the signs of the patient and paying attention to the unexplained symptoms of the primary disease, the diagnosis of the disease can be confirmed by perfecting the relevant examination. At present, glucocorticoid combined with immunosuppressant is often used in the treatment of this disease.
【学位授予单位】：浙江大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R544.1;R597

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