本文選題：木村病 + 病理�。� 參考：《吉林大學》2017年碩士論文

【摘要】：背景:木村病(kimura’s disease,KD)是以長期存在并易反復的無痛性皮下軟組織腫物為主要臨床表現(xiàn)的一種病因尚未明確的慢性炎癥性疾病。本病發(fā)病率低,臨床較少見,近20年國內外相關病例報道僅近千例,好發(fā)于亞裔人群,且因木村病的臨床表現(xiàn)缺乏特異性,臨床上極易被漏診、誤診。目前木村病常用的治療方式包括手術切除、糖皮質激素治療、化學治療、局部放療,但均易復發(fā);而對聯(lián)合治療能否減少復發(fā)的研究較少,各學者在治療方案的選擇上尚有分歧。目的:通過分析相關文獻報道,總結木村病的臨床特點及診治要點,并對比各治療方案預后情況,從而加深臨床醫(yī)師對木村病的認識,減少誤診、漏診的發(fā)生,并尋求最佳治療方案,減少復發(fā)。方法:檢索2000年1月至2017年3月為止公開發(fā)表的KD病例國內外文獻,篩選相關病例。對入選病例的基本資料、臨床表現(xiàn)、相關并發(fā)癥、實驗室檢查、影像學檢查、病理檢查、治療隨訪情況進行歸納總結。結果:篩選入組的297例經病理檢查確診為KD的患者中,男性244例,女性53例,男女比例4.6:1。發(fā)病年齡6個月—75歲,平均年齡34.2歲,病程1周至45年不等;其中亞裔人285例。297例患者就診原因中以85.8%(254/297)無痛性頭頸部皮下腫物為主;其中3.4%(10/297)并發(fā)腎病綜合征。實驗室檢查中86.7%(196/226)外周血嗜酸性粒細胞升高;77.9%(74/95)患者血清Ig E水平升高;11.7%(16/137)尿蛋白檢測呈陽性。經治療達到部分緩解(PR)或完全緩解(CR)后,65.5%(19/29)血清Ig E水平降至正常范圍,27.6%(8/29)降低50%以上;90.2%(37/41)外周血嗜酸性粒細胞降至正常水平。手術聯(lián)合糖皮質激素治療組與手術聯(lián)合糖皮質激素、局部放療組較單純手術治療組復發(fā)率明顯降低;糖皮質激素治療組復發(fā)率高于單純手術治療、局部放療及聯(lián)合治療方案;局部放療組與單純手術治療組復發(fā)率無明顯差異,但低于糖皮質激素聯(lián)合免疫抑制劑治療組;單純手術治療組與糖皮質激素聯(lián)合免疫抑制劑治療組無明顯差異。結論:1.對于頭頸部無痛性皮下腫物的患者,應警惕木村病的可能;而木村病在診斷上主要依據其病理檢查,結合其臨床特點及實驗室檢查可確診。2.外周血嗜酸性粒細胞及血清Ig E水平可成為木村病患者診斷、療效、預后評估及隨訪監(jiān)測的一項指標。3.木村病患者在診治及隨訪過程中,應關注有無腎病綜合征的相關臨床表現(xiàn)并行相關檢查,以期做到早期診治。4.木村病治療首選手術治療,加用糖皮質激素治療可減少術后復發(fā);當患者無法行手術治療時,推薦使用局部放療;不建議單用糖皮質激素治療,不建議糖皮質激素聯(lián)合免疫抑制劑治療。
[Abstract]:Background: kimura disease (KD) is a chronic inflammatory disease which is characterized by chronic painless subcutaneous soft tissue mass.The incidence of this disease is low, clinical is relatively rare, in recent 20 years domestic and foreign related cases reported only about 1000 cases, prone to occur in Asian people, and because of the lack of specificity of the clinical manifestations of Kimura disease, it is easy to be missed clinically, misdiagnosed.At present, the commonly used treatments for Kimura disease include surgical resection, glucocorticoid therapy, chemotherapeutic therapy, and local radiotherapy, but all of them are prone to relapse; however, there are few studies on whether combined therapy can reduce recurrence.There are still differences among scholars on the choice of treatment plan.Objective: to summarize the clinical characteristics, diagnosis and treatment of Kimura disease by analyzing the related literature, and to compare the prognosis of each treatment plan, so as to deepen the clinicians' understanding of the disease and reduce the occurrence of misdiagnosis and missed diagnosis.And to seek the best treatment to reduce recurrence.Methods: the literatures of KD cases published from January 2000 to March 2017 were searched and selected.The basic data, clinical manifestation, related complications, laboratory examination, imaging examination, pathological examination and treatment follow-up were summarized.Results: of 297 selected patients, 244 were male and 53 were female, the ratio of male to female was 4.6: 1.The onset age ranged from 6 months to 75 years with an average age of 34.2 years and the course of disease ranged from 1 week to 45 years.In laboratory examination, 86.7% of eosinophils in peripheral blood increased 77.9% and 74% of 95%) the level of serum IgE increased 11.7% 16 / 137) urinary protein was positive.After treatment, the level of serum IgE decreased to a normal range of 27.6% and 8 / 29) after treatment with partial remission (PR) or complete remission (CRR), the level of eosinophils in peripheral blood decreased to normal level by more than 50% (90.22 / 41).The recurrence rate of operation combined with glucocorticoid group and surgery combined with glucocorticoid group was significantly lower than that of simple operation group, and the recurrence rate of glucocorticoid group was higher than that of simple operation treatment group, local radiotherapy group and combined treatment plan.The recurrence rate of local radiotherapy group was not significantly different from that of simple operation group, but lower than that of glucocorticoid combined with immunosuppressant group, and there was no significant difference between simple operation group and glucocorticoid combined immunosuppressant group.Conclusion 1.Patients with painless subcutaneous masses of head and neck should be on guard against the possibility of Kimura disease, and the diagnosis of Kimura disease is mainly based on its pathological examination, combined with its clinical characteristics and laboratory examination can be diagnosed. 2.The levels of eosinophil and serum IgE in peripheral blood can be used as an index of diagnosis, curative effect, prognosis evaluation and follow-up monitoring in patients with Kimura disease.In the course of diagnosis, treatment and follow-up, the patients with Kimura disease should pay attention to the related clinical manifestations of nephrotic syndrome and correlation examination, in order to achieve early diagnosis and treatment. 4.The first choice for the treatment of Kimura disease is surgical treatment, and the addition of glucocorticoid therapy can reduce postoperative recurrence; when the patient cannot be treated with surgery, local radiotherapy is recommended; and only glucocorticoid therapy is not recommended.Glucocorticoid combined with immunosuppressant therapy is not recommended.
【學位授予單位】：吉林大學
【學位級別】：碩士
【學位授予年份】：2017
【分類號】：R597

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