本文選題：POEMS綜合征 + 肺動脈高壓��； 參考：《浙江大學》2017年碩士論文

【摘要】：背景與目的:肺動脈高壓是指肺動脈壓力超過一定界限的一種血流動力學異常狀態(tài),本身并非一種獨立的疾病,它即可來源于肺血管本身的病變,也可繼發(fā)于其他系統(tǒng)疾病。POEMS綜合征又稱為Crow-Fukase綜合征,是一種可累及皮膚、淋巴結(jié)、肝、脾、肺、腎、內(nèi)分泌系統(tǒng)、神經(jīng)系統(tǒng)及血液系統(tǒng)等多器官、多系統(tǒng)的漿細胞異常增生性疾病�；颊叩呐R床癥狀往往缺乏特異性,可并發(fā)包括肺動脈高壓在內(nèi)的多種并發(fā)癥。POEMS綜合征合并肺動脈高壓的患者的臨床表現(xiàn)多樣,起病緩急不一,對臨床醫(yī)生診斷本病帶來許多困難,臨床誤診、漏診率極高。本文通過2例POEMS綜合征合并肺動脈高壓患者的臨床報道,并收集同時期我國文獻報道的病例,分析總結(jié)該類疾病的臨床特點、輔助檢查、治療及轉(zhuǎn)歸等特點,以提高對該疾病的認識。方法:采用病例檢索系統(tǒng),收集2013年1月至2017年1月在浙江大學醫(yī)學院附屬邵逸夫醫(yī)院住院診治的POEMS綜合征合并肺動脈高壓病例。并以"POEMS綜合征"和"肺動脈高壓"為關(guān)鍵詞檢索數(shù)據(jù)庫在相同時期報道的我國病例,收集其臨床特點、輔助檢查、治療及轉(zhuǎn)歸等資料,結(jié)合文獻進行分析。結(jié)果:病例共計6例,男性1例,女性5例,年齡分布為35-74歲,平均年齡為52.33歲。6例患者的主要臨床癥狀為氣急、外周水腫、四肢麻木、皮膚改變,乏力等,部分患者有納差,口干,咳嗽等癥狀。實驗室檢查中多數(shù)患者存在血沉增快、血氧飽和度降低、甲狀腺功能減退等情況,僅有少數(shù)出現(xiàn)血常規(guī)異常。影像學檢查提示最為多見的陽性結(jié)果為胸腔積液、心包積液、多發(fā)淋巴結(jié)腫大、脾大,部分患者存在肝大、腹水等表現(xiàn)。所有患者均存在M蛋白血癥和周圍神經(jīng)病變,2例經(jīng)淋巴結(jié)活檢病理結(jié)果提示存在Castleman病,1例患者并發(fā)了慢性腎衰竭,1例患者還并發(fā)了男性乳房發(fā)育。6例患者從起病到明確診斷的時間平均為5年,最短2個月,最長達16年。6例患者在治療后臨床癥狀都得到明顯緩解,出院后2例患者失訪,1例隨訪2年后死亡,3例隨訪至今。結(jié)論:POEMS綜合征合并肺動脈高壓患者臨床誤診率極高,其臨床表現(xiàn)、病程進展、輔助檢查結(jié)果具有多樣性,常出現(xiàn)漏診與誤診。相較于通常的肺動脈高壓患者,POEMS綜合征合并肺動脈高壓患者還常伴有皮膚改變、臟器腫大、周圍神經(jīng)病、M蛋白血癥、內(nèi)分泌異常、血管內(nèi)皮生長因子(vascular endothelial growth factor,VEGF)升高等表現(xiàn)。在詳細詢問病史,充分關(guān)注患者的體征,對于原發(fā)病難以解釋的癥狀加以關(guān)注后,完善相關(guān)檢查可明確診斷本病。目前臨床上常采用糖皮質(zhì)激素聯(lián)合免疫抑制劑治療本病,可獲得較明顯的治療效果。
[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.
【學位授予單位】：浙江大學
【學位級別】：碩士
【學位授予年份】：2017
【分類號】：R544.1;R597

【參考文獻】

相關(guān)期刊論文 前7條

1 成瑋;蔣瑛;張維佳;李曉華;徐艷紅;貝鵬劍;;以甲狀腺功能減退癥合并全身性水腫、肺動脈高壓為首發(fā)表現(xiàn)的POEMS綜合征1例報道[J];上海交通大學學報(醫(yī)學版);2017年01期

2 徐艷紅;李曉華;貝鵬劍;;一例POEMS綜合征伴重度肺動脈高壓病例報道[J];世界臨床藥物;2016年10期

3 黎金玲;侯黎莉;孫春燕;嚴蕾;劉錦銘;;POEMS綜合征合并肺動脈高壓右心衰竭1例護理[J];上海護理;2014年06期

4 萬萍;鐘兵;;以皮膚色素沉著、紅斑、結(jié)節(jié)及潰瘍?yōu)槭装l(fā)表現(xiàn)的POEMS綜合征1例[J];第三軍醫(yī)大學學報;2013年13期

5 張育安;羅長青;朱紅艷;王玉梅;張春;劉建社;易利霞;;POEMS綜合征并TMA樣腎損害一例[J];臨床腎臟病雜志;2013年03期

6 劉淑芬;朱衛(wèi)國;曾學軍;許文兵;;POEMS綜合征合并肺動脈高壓臨床分析[J];基礎(chǔ)醫(yī)學與臨床;2011年05期

7 曾杰;張磊;李蓬秋;;POEMS綜合征伴肺動脈高壓一例[J];中華內(nèi)科雜志;2010年02期

，

本文編號：1923534