發(fā)布時間：2018-05-15 23:28

  本文選題：嗜酸性粒細(xì)胞 + 原因不明�。� 參考：《廣西醫(yī)科大學(xué)》2017年碩士論文

【摘要】：目的:探討慢性嗜酸細(xì)胞性肺炎(CEP)的臨床診療過程及其特征,提高對CEP的認(rèn)識。方法:回顧性分析2008年1月至2016年9月廣西醫(yī)科大學(xué)第一附屬醫(yī)院收治的經(jīng)實(shí)驗(yàn)室檢驗(yàn)、結(jié)合影像資料及病理結(jié)果最終診斷原因尚不清楚的4例慢性嗜酸性肺炎患者的臨床資料,并檢索中知、萬方、Pubmed等數(shù)據(jù)庫,對相關(guān)文獻(xiàn)進(jìn)行系統(tǒng)性分析,歸納其臨床特點(diǎn)。結(jié)果:(1)我院病例資料:(1)4例患者,男:女1:1,其中有吸煙史2例,年齡26~67歲,病程3月~4年。起病亞急性或慢性,無肺外其他系統(tǒng)癥狀,4例均有咳嗽,咳少量白痰、活動后氣促2例,喘息、胸痛、胸悶各1例,發(fā)熱、乏力、體重下降各1例。1例聞及濕Up音。(2)4例患者均行自身抗體譜、抗鏈球菌溶血素O、血沉、補(bǔ)體、常規(guī)生化、肌酶譜、寄生蟲、病原學(xué)、腫瘤抗原、T細(xì)胞亞群等檢查,并結(jié)合病史,排除了繼發(fā)性EOS增多、肺以外器官系統(tǒng)EOS浸潤和伴有EOS增多的其他間質(zhì)性肺病。4例患者曾全部誤診,分別為“支氣管哮喘、支氣管炎、細(xì)菌性肺炎、肺結(jié)核”,起病1月~2年后才確診。(3)3例患者外周血EOS增高(1.01~3.34×10~9/L),2例外周血白細(xì)胞總數(shù)升高、輕度貧血、低氧血癥,3例ESR增快、4例CRP升高,1例骨髓細(xì)胞學(xué)見成熟嗜酸細(xì)胞增多、未見原始細(xì)胞,F/P融合基因陰性。(4)4例纖支鏡鏡下均可見支氣管粘膜充血,2例管腔內(nèi)見膿性分泌物。2例行肺泡灌洗液細(xì)胞分類計數(shù)檢查,分別為EOS比例增高(30%)、EOS計數(shù)升高(0.0217×10~9/L)。4例tblb均見較多eos浸潤,亦見到伴淋巴細(xì)胞浸潤,其中2例肺間質(zhì)腫脹、肺泡上皮輕度增生。(5)肺ct示磨玻璃影4例,斑片狀、條索狀實(shí)變影3例,不均勻斑片狀致密影、空洞、病灶游走各2例。4例均為雙肺受累,3例以上肺為主,3例病變主要位于肺周邊、胸膜下。(6)3例患者做肺功能檢查,彌散障礙2例,混合性以阻塞為主通氣障礙1例,通氣功能正常2例。(7)4例均口服糖皮質(zhì)激素治療,療程2月~4年,1周內(nèi)癥狀改善,1~2周外周血eos恢復(fù)正常,1周~2月復(fù)查肺ct病灶明顯吸收,后續(xù)隨訪中3例完全吸收,另1例激素減量過程中復(fù)發(fā)2次、病灶游走、但總體上明顯吸收。僅1例復(fù)查肺功能,提示彌散功能好轉(zhuǎn)。(2)中英文文獻(xiàn)復(fù)習(xí)結(jié)果:1975年至今僅70例報道資料較完整,肺部有陰影、balf或肺活檢均證實(shí)eos浸潤,且排除繼發(fā)性、腫瘤、自身免疫性疾病、肺外器官eos浸潤的疾病及其他間質(zhì)性肺病后診斷慢性嗜酸性肺炎,多為個案報道。(1)年齡跨度大(1~79歲),平均年齡50.13歲,40~69歲為高峰,僅3例有吸煙史,男:女26:35。19例確診前有誤診,14例既往有哮喘病史。僅1例急性起病,余亞急性或慢性,無肺外其他系統(tǒng)癥狀,以喘息、氣促、咳痰,伴發(fā)熱、盜汗、體重下降為主要表現(xiàn);僅3例有急性呼吸衰竭、需機(jī)械通氣,22例肺部有Up音、1例有杵狀指。(2)87.7%外周血eos比例升高,8例骨髓均見成熟eos增多,未見幼稚細(xì)胞,分別有1例提供f/p、jak2基因結(jié)果提示陰性,1例染色體核型正常。54例做纖支鏡檢查,8例鏡下見粘液痰栓、2例見支氣管壁白色小結(jié)節(jié),balf中eos比例全部升高(平均49.1%)。50例肺活檢均可見到肺泡、肺間質(zhì)eos浸潤,6例見輕度纖維化、機(jī)化。34例提供肺功能結(jié)果,彌散障礙68.4%,通氣功能可呈阻塞性、限制性、混合性或正常,其中阻塞性47.1%、較多見于既往有哮喘病史患者。(3)60%以上患者影像學(xué)表現(xiàn)為雙上肺、外周分布為主、多發(fā)實(shí)變、磨玻璃影,13.4%見病灶游走。(4)確診后除4例以單一ics作為初始治療外,66例患者口服相當(dāng)于強(qiáng)的松0.3~2.0mg/kg.d開始治療,其中62例為0.5~1.0mg/Kg.d,療程平均9月,最長11年。4例單一ICS治療患者病情進(jìn)展后改用口服激素治療癥狀及影像學(xué)均改善。(5)預(yù)后良好,僅1例因冠心病突發(fā)死亡,激素減量或停藥復(fù)發(fā)有12次,8次見于開始治療1月~6月停藥患者。復(fù)發(fā)后重新激素治療均再次好轉(zhuǎn)。結(jié)論:(1)嗜酸細(xì)胞浸潤肺組織,經(jīng)過常規(guī)生化、病原學(xué)、免疫抗原抗體等檢查,排除繼發(fā)因素、無肺外器官累及,原因尚不明確,需考慮慢性嗜酸細(xì)胞性肺炎可能。(2)骨髓融合基因檢測有助于排除克隆性骨髓增殖性疾病,其在慢性嗜酸細(xì)胞性肺炎的診斷程序中需要更多的研究進(jìn)一步探討。(3)慢性嗜酸細(xì)胞性肺炎病程長、預(yù)后良好,40~69歲為發(fā)病高峰年齡,以咳嗽、氣促喘息為主要表現(xiàn),可伴發(fā)熱、盜汗、體重下降等全身癥狀,無胸外癥狀、易誤診。(4)影像學(xué)超過60%患者表現(xiàn)為雙上肺、外周分布為主的多發(fā)斑片狀實(shí)變、磨玻璃影,16.6%見病灶游走。(5)首選口服糖皮質(zhì)激素治療,建議強(qiáng)的松0.5~1mg/kg.d為初始劑量,療程9月左右,不應(yīng)少于6月,6月內(nèi)停藥或激素減量易復(fù)發(fā),復(fù)發(fā)后激素仍然敏感,可酌情減慢減量過程、延長療程。
[Abstract]:Objective: To explore the clinical diagnosis and treatment process of chronic eosinophilic pneumonia (CEP) and its characteristics, and to improve the understanding of CEP. Methods: a retrospective analysis of 4 cases of chronic acidophilia in the First Affiliated Hospital of Guangxi Medical University from January 2008 to September 2016, combined with the image data and the final diagnosis of pathological results, was not clear. The clinical data of the pneumonia patient, and retrieved knowledge, Wanfang, Pubmed and other databases, systematically analyzed the related literature, and summed up its clinical characteristics. Results: (1) the case data of our hospital: (1) 4 cases, male: female 1:1, among them, there were 2 cases of smoking history, age 26~67 years, the course of disease in March ~4. The onset of subacute or chronic disease, no other systemic symptoms of the lung, 4 cases were all There were 2 cases of cough, cough, white sputum, 2 cases of breath, chest pain, chest tightness in 1 cases, fever, fatigue, and weight loss in 1 cases of.1 cases and wet Up sound. (2) all 4 patients received autoantibody spectrum, anti Streptococcus hemolysin O, erythrocyte sedimentation, complement, routine biochemistry, muscle enzyme spectrum, parasite, etiological, tumor antigen, T cell subgroup, etc. In addition to secondary EOS increase, EOS infiltration of the organ system outside the lung and other interstitial lung diseases with EOS increase in.4 were all misdiagnosed as "bronchial asthma, bronchitis, bacterial pneumonia, pulmonary tuberculosis", and the disease was confirmed only after ~2 years in January. (3) 3 patients had increased peripheral blood EOS (1.01~3.34 x 10~9/L), and 2 cases of peripheral leukocyte total. Increase in number, mild anemia, hypoxemia, 3 cases of ESR faster, 4 cases of CRP increase, 1 cases of bone marrow cytology to see more eosinophil, no original cells, F/P fusion gene negative. (4) 4 cases of bronchoscopy under the bronchoscopy can show the congestion of bronchial mucosa, 2 cases of purulent secretory substance in the tube.2 routine alveolar lavage cell classification count, respectively, EOS ratio, respectively. Cases increased (30%), EOS count increased (0.0217 x 10~9/L).4 cases, TBLB showed more EOS infiltration and also accompanied by lymphocytic infiltration, including 2 cases of pulmonary interstitial swelling and mild hyperplasia of alveolar epithelium. (5) 4 cases of lung CT grind glass shadow, 3 cases of patchy, streaklike solid shadow, nonuniform patch shape density shadow, cavity, and 2 cases of.4 cases in each of the lesions, each of the lesions, all.4 cases were double lung involvement, 3. 3 Above lung, 3 cases were mainly located in the periphery of the lung, under the pleura. (6) 3 patients had pulmonary function examination, 2 cases of diffusion barrier, 1 cases with obstructive ventilatory obstruction and 2 cases of normal ventilation. (7) 4 cases were treated with glucocorticoid, the course of treatment was improved in ~4 years in February, the symptoms were improved in 1 weeks, and the peripheral blood EOS was restored to normal in 1~2 weeks, and CT of 1 weeks ~2 month was rechecked CT lung CT. Lung CT was rechecked 1 weeks lung CT lung CT check lung CT review lung CT check lung CT review lung CT for ~2 month reexamination lung CT The lesions were absorbed obviously, 3 cases were completely absorbed in follow-up and 2 times in 1 cases of hormone reduction, and the lesion wandering, but overall obvious absorption. Only 1 cases rechecked pulmonary function, suggesting that diffusion function improved. (2) the literature review in Chinese and English Literature: from 1975 to date, only 70 cases were reported to be more complete, lung had shadow, BALF or lung biopsy confirmed EOS immersion Moistening, and excluding secondary, tumor, autoimmune disease, EOS infiltration of extrapulmonary organs and other interstitial lung disease diagnosis of chronic eosinophilic pneumonia. (1) the age span is large (1~79 years old), the average age is 50.13 years old, 40~69 is the peak, only 3 cases have smoking history, male: 26:35.19 cases have misdiagnosis before diagnosis, 14 cases have wheeze. The history of wheezing. Only 1 cases of acute onset, Yu Ya acute or chronic, no other systemic symptoms of lung, breathing, breath, expectoration, fever, sweating, weight loss, only 3 cases with acute respiratory failure, mechanical ventilation, 22 cases of Up in the lungs, 1 cases of clubbing. (2) 87.7% peripheral blood EOS ratio increased, 8 cases of bone marrow and mature EOS increased, and no bone marrow. There were 1 cases of naive cells, 1 cases were provided with f/p, JAK2 gene results were negative, 1 cases of chromosome karyotype normal.54 cases, 8 cases with mucous phlegm thrombus, 2 cases of white small nodules in bronchial wall, and all EOS ratio in BALF (mean 49.1%),.50 cases of lung biopsy showed pulmonary alveolus, pulmonary interstitial EOS infiltration, 6 case mild fibrosis, machine .34 cases provided the results of pulmonary function, diffusion barrier 68.4%, ventilation function can be obstructive, restrictive, mixed or normal, of which obstructive 47.1%, more frequently seen in patients with past history of asthma. (3) more than 60% of the patients were characterized by double upper lung, peripheral distribution, multiple real changes, glass shadow, 13.4% lesions wandering. (4) 4 cases (4) 4 cases except 4 cases except 4 cases, except 4 cases after diagnosis except after diagnosis except 4 cases except 4 cases except 4 cases except after diagnosis after diagnosis. With a single ICs as the initial treatment, 66 patients were given the equivalent of prednisone 0.3~2.0mg/kg.d, of which 62 cases were 0.5~1.0mg/Kg.d, the average course of treatment was September, the longest 11 years of.4 patients with single ICS treatment were improved by oral hormone treatment and imaging. (5) the prognosis was good, only 1 cases died of CHD sudden death. There were 12 times of reduced or stopped drug recurrence, 8 times in the beginning of the treatment of ~6 months in January. After relapse, re hormone treatment was all better again. Conclusion: (1) eosinophil infiltration of lung tissue, after routine biochemical, pathogenic, immuno antigen antibody examination, excluding secondary factors, no external pulmonary organ involvement, the reason is not clear, need to consider chronic eosinophilia. Cytosolic pneumonia may. (2) bone marrow fusion gene detection helps to exclude cloned myeloproliferative diseases, and it needs further study in the diagnosis of chronic eosinophilic pneumonia. (3) chronic eosinophilic pneumonia has a long course, good prognosis, 40~69 age as the peak age, cough and gaspant breath as the main table. Now, it can be accompanied by fever, night sweating, weight loss and other systemic symptoms, without external symptoms of chest, easy to be misdiagnosed. (4) more than 60% of the patients with imaging findings are double upper lung, the peripheral distribution of multiple patchy changes, glass shadow and 16.6% of the lesions travel. (5) the first choice of oral glucocorticoid therapy, recommended prednisone 0.5~1mg/kg.d as the initial dose, about September, course of treatment, It should not be less than June. In June, drug withdrawal or hormone reduction is easy to relapse. Hormone is still sensitive after relapse.

【學(xué)位授予單位】：廣西醫(yī)科大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2017
【分類號】：R563.1

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本文編號：1894431