發(fā)布時間：2018-02-19 19:35

  本文關(guān)鍵詞： 嗜酸性粒細(xì)胞增多綜合征 嗜酸性粒細(xì)胞增多性皮炎 發(fā)病率 分型 治療　出處：《山東大學(xué)》2012年碩士論文　論文類型：學(xué)位論文

【摘要】：目的：分析HES的臨床特點(diǎn),探討該病的治療方法,分析該病的預(yù)后,對該病的診斷及治療提供幫助。 方法：回顧性分析1例確診為HED的患者的臨床資料；檢索自2000年～2011年發(fā)表在CNKI、Pubmed、CBM等數(shù)據(jù)庫中的國內(nèi)外關(guān)于HES的病例報道,并重點(diǎn)對國內(nèi)近10年報道的關(guān)于HES的53篇文獻(xiàn)中資料較完整的46例患者進(jìn)行分析,收集報道病例的性別、年齡、臨床表現(xiàn)、病程、輔助檢查、治療方案及預(yù)后等一般資料,分析我國HES的病因、發(fā)病機(jī)制、臨床表現(xiàn)、實(shí)驗(yàn)室檢查、組織病理檢查及骨髓細(xì)胞學(xué)檢查等,與國外相關(guān)報道進(jìn)行比較,對我國HES的好發(fā)人群、發(fā)病年齡、臨床特點(diǎn)、并發(fā)癥、治療、預(yù)后及與其他合并嗜酸性粒細(xì)胞增多的疾病的聯(lián)系與區(qū)別等問題進(jìn)行進(jìn)一步的分析。 結(jié)果：HES病因不明,臨床表現(xiàn)復(fù)雜,可伴有多系統(tǒng)損害及出現(xiàn)各種各樣皮疹,首診常易誤診,預(yù)后有較大差異。 (1)我們報道的該病例為淋巴細(xì)胞型HES,臨床表現(xiàn)以皮膚受累為主,對糖皮質(zhì)激素治療反應(yīng)較好,甲磺酸伊馬替尼對該患者有一定療效。目前該患者仍在隨訪之中。 (2)復(fù)習(xí)相關(guān)文獻(xiàn)報道的46例患者中,男性34例,女性12例,男女發(fā)病率之比約為3：1。 (3)年齡：最小2歲,最大92歲,中位發(fā)病年齡36歲,平均發(fā)病年齡39歲。其中8例小于18歲,19例大于40歲。 (4)46例患者中,21例出現(xiàn)皮疹,19例發(fā)熱,11例伴有明顯瘙癢癥狀,11例出現(xiàn)胸悶、咳嗽、咯痰及胸痛,10例出現(xiàn)頭暈、乏力,8例出現(xiàn)腹痛、腹瀉,2例累及腎臟,1例出現(xiàn)消瘦。 (5)皮膚、骨髓及心臟、肺部、腹部、神經(jīng)系統(tǒng)均可受累。 (6)國內(nèi)外HES在發(fā)病年齡及系統(tǒng)受累等方面無明顯差異。我國發(fā)病率男女之比約為3：1,與國外報道9：1相差較大。M-HES好發(fā)于中老年男性,L-HES無明顯性別差異。 (7)實(shí)驗(yàn)室檢查：FIP1L1-PDGFRA融合基因陽性率在10%～15%；IgE或其他免疫球蛋白水平升高：胸腺和活化調(diào)節(jié)因子(TARC)水平升高；異常T細(xì)胞亞群；IL-2、IL-3、IL-5及GM-CSF等細(xì)胞因子水平升高；TCR基因重排部分病歷可發(fā)現(xiàn)單克隆性淋巴系統(tǒng)疾病。 (8)免疫分型：分為骨髓型和淋巴細(xì)胞型兩型；M-HES可有多種染色體異常；L-HES也可存在染色體異常。 (9)骨髓型HES常有血液學(xué)異常,包括血小板減少,貧血,骨髓纖維化,F/P融合基因,染色體異常,高免疫球蛋白血癥等,有轉(zhuǎn)化為CEL的風(fēng)險,病程惡性,預(yù)后不好,死亡率較淋巴型高。淋巴型HES常以皮膚受累表現(xiàn)為主,有轉(zhuǎn)化為淋巴瘤的可能,應(yīng)予密切隨訪。 (10)治療以糖皮質(zhì)激素、細(xì)胞毒藥物為主；可以使用干擾素；甲磺酸伊馬替尼對FIP1L1-PDGFRA (+)患者效果最好,對40%FIP1L1-PDGFRA(-)可能有效；美泊利單抗(IL-5的人源化抗體)和SCH55700單抗,能與IL-5結(jié)合,從而阻斷IL-5與嗜酸性粒細(xì)胞表面上IL-5受體的相互作用；白介素2受體對特定HES有效；體外光化學(xué)療法可能有效；有報道使用自體造血干細(xì)胞移植對嗜酸性粒細(xì)胞增多綜合征(HES)有效。 結(jié)論：隨著對該病認(rèn)識的逐漸深入,更傾向認(rèn)為該病系一組病譜性疾病,病譜的一端為良性疾病,即僅累及皮膚的嗜酸性粒細(xì)胞增多性皮炎(HED),另一端為病程惡性的慢性嗜酸性粒細(xì)胞白血病(CEL),預(yù)后較差。F/P融合基因的發(fā)現(xiàn)及多種染色體異常支持HES為造血系統(tǒng)的惡性克隆性疾病,并可轉(zhuǎn)化為嗜酸性粒細(xì)胞白血病及淋巴瘤。該病臨床表現(xiàn)多樣,容易誤診、漏診,臨床診斷應(yīng)綜合分析患者病史、臨床表現(xiàn),并作血常規(guī)、骨髓細(xì)胞學(xué)檢查、F/P融合基因檢測、病理檢查、染色體檢查及免疫分型等,做到早診斷,個體化治療,改善預(yù)后,注意對患者進(jìn)行健康教育以及隨訪,預(yù)防疾病惡變,降低患者死亡率。
[Abstract]:Objective : To analyze the clinical characteristics of HES , discuss the treatment method of HES , analyze the prognosis of the disease , and provide help for the diagnosis and treatment of the disease . Methods : The clinical data of the patients diagnosed as HED were analyzed retrospectively . The cases of HES were analyzed from 2000 to 2011 in CNKI , Pubmed , CBM , etc . The etiology , pathogenesis , clinical manifestation , disease course , auxiliary examination , treatment plan and prognosis of HES were analyzed . Results : The etiology of HES is unknown , the clinical manifestation is complicated , it can be accompanied by multiple systemic damage and various rash , the first diagnosis is often misdiagnosed , and the prognosis is significantly different . ( 1 ) We reported that the case was lymphocyte - type HES , the clinical manifestations were mainly skin - affected , the response to glucocorticoid therapy was better , and imatinib mesylate had a certain curative effect on the patient . ( 2 ) Of the 46 cases reported in the related literature , 34 cases were male , 12 were female , and the ratio of male to female was about 3 : 1 . ( 3 ) Age : minimum 2 years , maximum 92 years , median onset age 36 years , average onset age 39 years . Among them 8 cases were younger than 18 years old , 19 cases were more than 40 years old . ( 4 ) Of the 46 cases , 21 cases had rash , 19 cases had fever , 11 cases had obvious itching symptoms , 11 cases had chest distress , cough , sputum and chest pain , 10 cases had dizziness , asthenia , 8 cases had abdominal pain , diarrhea , 2 cases involving kidney , 1 case was thin . ( 5 ) The skin , bone marrow and heart , lung , abdomen and nervous system can be affected . ( 6 ) There was no significant difference in the age of onset and system involvement of HES at home and abroad . The ratio of male and female in China was about 3 : 1 , which differed significantly from 9 : 1 reported abroad . M - HES was good for middle - aged and old men , and L - HES had no obvious sex difference . ( 7 ) Laboratory examination : The positive rate of FIP1L1 - PDGFRA fusion gene was 10 % ~ 15 % ; the level of IgE or other immunoglobulin increased : the level of thymus and activation regulating factor ( TARC ) increased ; abnormal T cell subgroup ; IL - 2 , IL - 3 , IL - 5 , GM - CSF and other cytokines were elevated ; TCR gene rearrangement partial medical record could find monoclonal lymphatic system disease . ( 8 ) Immuntyping : There are two types : bone marrow type and lymphocyte type ; M - HES may have multiple chromosomal abnormalities ; L - HES may also have chromosome abnormalities . ( 9 ) Bone marrow - type HES has abnormal hematology abnormalities , including thrombocytopenia , anemia , bone marrow fibrosis , F / P fusion gene , chromosomal abnormality , high immunoglobulin , etc . ( 10 ) Treatment with glucocorticoid and cytotoxic drugs ; the effect of imatinib mesylate on FIP1L1 - PDGFRA ( + ) patients is the best , which may be effective for patients with FIP1L1 - PDGFRA ( + ) ; the interaction of IL - 5 with IL - 5 receptors on eosinophils can be blocked ; the interleukin 2 receptor is effective for specific HES ; in vitro photochemical therapy may be effective ; it is reported that the use of autologous hematopoietic stem cell transplantation is effective for eosinophils . Conclusion : With the deeper understanding of the disease , it is more apt to think that the disease is a group of diseases , one end of the disease spectrum is benign disease , the other end is the malignant clonal disease of the hematopoietic system , and the other end is the malignant clonal disease of the hematopoietic system . It can be used for early diagnosis , individual treatment , pathological examination , chromosome examination and immunological classification , etc . , and can be used for early diagnosis , individualized treatment , improvement of prognosis , and attention to the health education and follow - up of the patients , prevention of the malignant transformation of the disease and the reduction of the mortality rate of the patients .

【學(xué)位授予單位】：山東大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2012
【分類號】：R758.6

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