嗜酸性粒细胞增多性皮炎一例报道并嗜酸性粒细胞增多综合征文献复习

发布时间：2018-02-19 19:35

本文关键词： 嗜酸性粒细胞增多综合征嗜酸性粒细胞增多性皮炎发病率分型治疗　出处：《山东大学》2012年硕士论文　论文类型：学位论文

【摘要】：目的：分析HES的临床特点,探讨该病的治疗方法,分析该病的预后,对该病的诊断及治疗提供帮助。方法：回顾性分析1例确诊为HED的患者的临床资料；检索自2000年～2011年发表在CNKI、Pubmed、CBM等数据库中的国内外关于HES的病例报道,并重点对国内近10年报道的关于HES的53篇文献中资料较完整的46例患者进行分析,收集报道病例的性别、年龄、临床表现、病程、辅助检查、治疗方案及预后等一般资料,分析我国HES的病因、发病机制、临床表现、实验室检查、组织病理检查及骨髓细胞学检查等,与国外相关报道进行比较,对我国HES的好发人群、发病年龄、临床特点、并发症、治疗、预后及与其他合并嗜酸性粒细胞增多的疾病的联系与区别等问题进行进一步的分析。结果：HES病因不明,临床表现复杂,可伴有多系统损害及出现各种各样皮疹,首诊常易误诊,预后有较大差异。 (1)我们报道的该病例为淋巴细胞型HES,临床表现以皮肤受累为主,对糖皮质激素治疗反应较好,甲磺酸伊马替尼对该患者有一定疗效。目前该患者仍在随访之中。 (2)复习相关文献报道的46例患者中,男性34例,女性12例,男女发病率之比约为3：1。 (3)年龄：最小2岁,最大92岁,中位发病年龄36岁,平均发病年龄39岁。其中8例小于18岁,19例大于40岁。 (4)46例患者中,21例出现皮疹,19例发热,11例伴有明显瘙痒症状,11例出现胸闷、咳嗽、咯痰及胸痛,10例出现头晕、乏力,8例出现腹痛、腹泻,2例累及肾脏,1例出现消瘦。 (5)皮肤、骨髓及心脏、肺部、腹部、神经系统均可受累。 (6)国内外HES在发病年龄及系统受累等方面无明显差异。我国发病率男女之比约为3：1,与国外报道9：1相差较大。M-HES好发于中老年男性,L-HES无明显性别差异。 (7)实验室检查：FIP1L1-PDGFRA融合基因阳性率在10%～15%；IgE或其他免疫球蛋白水平升高：胸腺和活化调节因子(TARC)水平升高；异常T细胞亚群；IL-2、IL-3、IL-5及GM-CSF等细胞因子水平升高；TCR基因重排部分病历可发现单克隆性淋巴系统疾病。 (8)免疫分型：分为骨髓型和淋巴细胞型两型；M-HES可有多种染色体异常；L-HES也可存在染色体异常。 (9)骨髓型HES常有血液学异常,包括血小板减少,贫血,骨髓纤维化,F/P融合基因,染色体异常,高免疫球蛋白血症等,有转化为CEL的风险,病程恶性,预后不好,死亡率较淋巴型高。淋巴型HES常以皮肤受累表现为主,有转化为淋巴瘤的可能,应予密切随访。 (10)治疗以糖皮质激素、细胞毒药物为主；可以使用干扰素；甲磺酸伊马替尼对FIP1L1-PDGFRA (+)患者效果最好,对40%FIP1L1-PDGFRA(-)可能有效；美泊利单抗(IL-5的人源化抗体)和SCH55700单抗,能与IL-5结合,从而阻断IL-5与嗜酸性粒细胞表面上IL-5受体的相互作用；白介素2受体对特定HES有效；体外光化学疗法可能有效；有报道使用自体造血干细胞移植对嗜酸性粒细胞增多综合征(HES)有效。结论：随着对该病认识的逐渐深入,更倾向认为该病系一组病谱性疾病,病谱的一端为良性疾病,即仅累及皮肤的嗜酸性粒细胞增多性皮炎(HED),另一端为病程恶性的慢性嗜酸性粒细胞白血病(CEL),预后较差。F/P融合基因的发现及多种染色体异常支持HES为造血系统的恶性克隆性疾病,并可转化为嗜酸性粒细胞白血病及淋巴瘤。该病临床表现多样,容易误诊、漏诊,临床诊断应综合分析患者病史、临床表现,并作血常规、骨髓细胞学检查、F/P融合基因检测、病理检查、染色体检查及免疫分型等,做到早诊断,个体化治疗,改善预后,注意对患者进行健康教育以及随访,预防疾病恶变,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 .

【学位授予单位】：山东大学
【学位级别】：硕士
【学位授予年份】：2012
【分类号】：R758.6

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