嗜酸性脂膜炎国内首例报告

报告国内首例嗜酸性脂膜炎。患者男,21岁,双下肢反复出现结节性红斑样皮疹5个月伴外周血嗜酸性粒细胞增高(3.8×10~9/L),组织病理检查示病变为混合型脂膜炎,脂膜内有大量嗜酸性粒细胞浸润。对本病的分类、鉴别诊断、治疗及其发病机理进行了讨论。     

嗜酸性粒细胞增多3例报告

嗜酸性粒细胞增多（即外周血嗜酸性粒细胞计数绝对值＞0.4×109/L[1]）常与寄生虫感染、过敏性疾病有关,但临床上病因多种多样.本文收集了2010-08~2011-03在我科住院伴有嗜酸性粒细胞增多3例进行临床分析如下.     

过敏性鼻炎患者血液嗜酸性粒细胞FcεRI和CD63表达研究

目的：检测过敏性鼻炎（allergic rhinitis,AR）患者外周血嗜酸性粒细胞FcεRI和CD63的表达。方法：收集AR患者和健康人（healthy controls,HCs）的外周血,用蒿草花粉和尘螨过敏原粗提液刺激,流式细胞仪检测嗜酸性粒细胞富集群中FcεRI和CD63的表达情况。结果：静息状态下,AR患者[0.92%（0.70%~1.64%）]相比于HCs[0.71%（0.43%~0.97%）]嗜酸性粒细胞比例上调（Z=-2.050,P=0.041）;AR患者[6.19%（3.05%,8.19%）;1 462（1 164,1 720）]FcεRI+嗜酸性粒细胞的比例和平均荧光强度（mean fluorescence intensity,MFI）比HCs[3.16%（1.55%,4.53%）;808（524,1308）]增加（Z=-2.102,P=0.036）;AR患者[2 031 （1 725,3 338）]CD63+嗜酸性粒细胞的MFI比HCs[1 491（1 297,1 613）]升高36%（Z=-3.192,P=0.002）。0.1 μg/mL蒿草过敏原粗提液[2 618（2 131,3 315）]和1.0 μg/mL蒿草过敏原粗提液[3 108（2 317,3 792）]能诱导AR患者CD63+嗜酸性粒细胞的MFI上调（Z=-2.667,P=0.008;Z=-2.845,P=0.004）。结论：嗜酸性粒细胞表达的FcεRI和CD63参与AR发病。过敏原特异性嗜酸性粒细胞激发试验检测CD63的表达,可能成为AR诊断的补充实验。     

嗜酸性粒细胞凋亡在鼻息肉发病机制中的作用

鼻息肉(nasal polyps,NP)是耳鼻咽喉科常见病,成人发病率为1%~2%,术后或保守治疗后复发率高。组织学特点为上皮增生、间质水肿、腺体水肿增生等改变,同时伴有炎性细胞浸润,以嗜酸性粒细胞浸润为主,尚可见淋巴细胞、浆细胞、中性粒细胞及巨噬细胞等。嗜酸粒细胞浸润增多是鼻息肉发病的关键,近年来有研究表明,鼻息肉组织中嗜酸性粒细胞凋亡抑制是导致组织中嗜酸性粒细胞浸润增多的机制之一。本文对其研究进展综述如下。1息肉中的嗜酸性粒细胞与细胞凋亡细胞凋亡(apoptosis)又称程序性细胞死亡(programmed celldeath,PCD),是由基因调控的细胞自主的有序性死亡。嗜酸粒细胞凋亡调节基因主要有Bcl家族蛋白和Fas/FasL系统。研究表明,鼻息肉组织中嗜酸性粒细胞凋亡受到抑制是引起嗜酸性粒细胞浸润增多的原因之一[1]。鼻息肉的发生与嗜酸性粒细胞的凋亡是复杂的多因素、多基因共同作用的结果,嗜酸性粒细胞在凋亡抑制或诱导的过程中,相关的凋亡调控基因表达可能会发生变化。1.1Bcl家族在鼻息肉中嗜酸性粒细胞的表达B细胞淋巴瘤/白血病(B-celllymphoma/leukemia,Bcl)家族蛋白是目前已...     

Loeffler心内膜炎伴血栓形成1例

<正>Loeffler感染性心内膜炎又名嗜酸性粒细胞增多性心内膜炎,是嗜酸性粒细胞增多累及心脏的表现。其临床表现多样,累及多个系统,可表现为发热、乏力、纳差、咳嗽、皮疹、进行性心衰、贫血、动脉栓塞、外周血及骨髓象示嗜酸性粒细胞增多[1]。本文将报道Loeffler心内膜炎引起心室血栓形成1例,为临床诊治提供帮助。     

嗜酸性粒细胞筋膜炎5例临床病理分析并文献复习

目的总结嗜酸性粒细胞筋膜炎(EF)的临床表现、病理特征和诊治方法。方法收集2017年4月—2018年9月武汉大学人民医院神经内科诊治的5例EF患者,均符合2014年Pinal-Femandez和2017年Masatoshi Jinnin嗜酸性粒细胞筋膜炎的诊断标准,对其危险因素、临床表现、实验室检查、肌电图、MR、病理组织活检表现及治疗进行分析、结果嗜酸性粒细胞筋膜炎主要表现为对称性的肢体肿胀、僵硬、疼痛,Groove征以及肌腱挛缩;实验室检查外周血嗜酸性粒细胞增加,高丙种球蛋白血症,红细胞沉降率和C反应蛋白升高,血清肌酸激酶正常;肌肉MR检查显示筋膜增厚;肌电图均表现为肌源性损害;全程皮肤、筋膜、肌肉连续活检,肉眼可见筋膜明显增厚,组织学检查显示5例患者皮肤及筋膜均以淋巴细胞为主的炎性细胞浸润,可见嗜酸性粒细胞,4例患者肌肉组织有炎性细胞浸润;激素治疗有明显疗效。结论嗜酸性粒细胞筋膜炎除皮肤和筋膜的表现外,还有肌肉受损的表现;诊断除临床、实验室、影像学等检查外,主要依靠活组织检查;早期诊断和激素治疗效果较好。     

嗜酸性筋膜炎一例报道

嗜酸性筋膜炎(eosinophilic fascitis,EF)是以筋膜炎症、胶原增殖、邻近的皮下脂肪组织与肌外膜等的纤维化为主要表现的慢性、全身性免疫疾病。临床上以皮肤疼痛性肿胀、躯干四肢皮肤结节、关节挛缩、血中嗜酸性粒细胞增多等为主要特征。本病较少见,近年来报道病例数逐年增多。如无系统受累,多数预后良好。     

嗜酸性粒细胞增多与临床

正常血液中嗜酸性粒细胞仅占粒细胞数比值0.005～0.05,绝对浓度数不超过0.5×109/L。在白介素-3(IL-3)、白介素-5(IL-5)和粒细胞/巨噬细胞集落刺激因子(GM-CFS)等细胞因子调节下,嗜酸性粒细胞具有重要的临床效应,嗜酸性粒细胞具有抗寄生虫作用,也可致炎作用使组织损伤。了解嗜酸性粒细胞增多的发病机制,有助于对嗜酸性粒细胞增多诊断与治疗。     

哮喘患儿血清IL-4、IL-13及嗜酸性粒细胞意义的探讨

[目的]检测哮喘患儿血清中IL-4、IL-13及嗜酸性粒细胞(EOS)的水平并探讨其临床意义。[方法]取中-重度哮喘患儿28例为实验组1、缓解期哮喘患儿24例为实验组2、取正常儿童25例为对照组,分别取外周静脉血3mL,用ELISA方法检测IL-4、IL-13的含量,并进行外周血嗜酸性粒细胞计数。[结果]两实验组IL-4、IL-13、EOS计数水平均高于对照组,实验组1较实验组2明显增高;且哮喘患儿血清IL-4与IL-13水平呈正相关。[结论]IL-4、IL-13及EOS参与了哮喘的发病过程,其水平反映了病情的严重程度;且这些因子间存在着一定联系。     

嗜酸性粒细胞在慢性鼻窦炎中的应用研究进展

慢性鼻 -鼻窦炎 (chronicrhinosinusitis ,CRS)是耳鼻喉科的常见病之一 ,其发病机制至今还未彻底清楚。许多组织病理研究证明CRS鼻窦黏膜组织中大量慢性炎症细胞浸润 ,其中嗜酸性粒细胞 (Eos)及其释放的毒性颗粒蛋白及炎性介质是引起鼻窦黏膜炎症发生、发展和迁延的重要原因之一[1] 。对Eos及其细胞因子的认识 ,成为研究CRS机制的热点之一。1　嗜酸性粒细胞及其毒性颗粒蛋白在CRS中的作用嗜酸性粒细胞 (Eos)不仅是变态反应的结果 ,更重要的是它有免疫调节作用。有观点认为CRS和鼻息肉是一种鼻黏膜的慢性的自体支持性的Eos性炎症[1] …     

踝部筋膜间隔综合征

笔者在临床实践中认为,踝部筋膜室综合征相似但又不同于小腿筋膜室综合征,且足部筋膜室综合征多并发于此征。踝部有三个骨筋膜室,即踝前筋膜室、踝后内侧筋膜室和踝后外侧筋膜室。笔者亦提出,早期诊断、早期切开筋膜室减压,以避免患足发生坏死。     

Pregnant lady with eosinophilia: common cause,uncommon association

Peripheral eosinophilia can have a myriad of causes and presents a diagnostic challenge in everyday practice. Tropical pulmonary eosinophilia (TPE), seen commonly in tropics, is an immunological reaction to filarial parasites. This disease can present with clinical features that closely mimic asthma, eosinophilic pneumonia, and Loffler's syndrome. Differentiating between these diseases is essential owing to marked differences in treatment. This can be further challenging in pregnancy as any wrong treatment is likely to affect both the mother and the child. We report a case of a pregnant lady who presented with eosinophilia and of how she was worked up to the correct diagnosis. There are only few reported cases of TPE in pregnancy, and there are no reported cases from India. The case also underlines the approach required in these patients to reach the correct diagnosis.     

Severe eosinophilia in disseminated gastric carcinoma

Peripheral blood eosinophilia is a well-recognised but unusual manifestation of malignancy, and may represent a paraneoplastic phenomenon. We present a case of poorly differentiated adenocarcinoma of the stomach associated with severe peripheral blood eosinophilia A 55-year old man was admitted for abdominal pain of one week duration. An incidental finding of leucocytosis with eosinophilia was noted. After excluding haematological and infectious causes, an oesophagogastroduodenoscopy (OGD) followed by biopsy confirmed the diagnosis. Eosinophilia appears to be a response to cytokine production,and treatment is aimed at the underlying malignancy, and reducing the eosinophil count when necessary, to prevent end-organ damage. Studies have shown that peripheral eosinophilia is associated with disseminated, metastatic disease and hence signifies a poor prognosis,whereas tissue eosinophilia in advanced cancer has a better survival rate.     

Dapsone syndrome--first Malaysian paediatric case report

Dapsone syndrome is a potentially fatal hypersensitivity reaction to sulphone. We report a 12-year-old girl who developed high grade fever associated with intense jaundice, exfoliative skin rash and hepatomegaly after five weeks of starting the multidrug regimen for the treatment of Hansen's disease. Laboratory investigations revealed presence of leucocytosis with eosinophilia, deranged liver enzymes and an abnormal coagulation profile. Immediate cessation of the offending drug and administration of steroid proved successful. A high level of clinical awareness is fundamental for early diagnosis of dapsone syndrome as initiation of a prompt treatment may lead to rapid recovery.     

手指高压油漆喷枪注射伤合并手筋膜室综合征的诊治分析

高压油漆喷枪直接喷射手指可导致喷射部位油漆注射伤并引起手筋膜室综合征。我院自2006年5月至2008年1月共收治手指高压油漆喷枪注射伤合并手筋膜室综合征6例,通过及时准确的术前和术后处理,患者得到及时的治疗,取得较好的预后,现报告如下。     

Eosinophilic enteritis presenting as surgical emergencies: a report of six cases

Eosinophilic enteritis is now commonly diagnosed in northern Queensland, and cases have been seen elsewhere. The patients present with acute abdominal symptoms, which may mimic appendicitis, and many cases are managed by surgery. To facilitate the diagnosis of this unusual syndrome, six surgically treated cases are reported here. Blood eosinophilia may be a helpful diagnostic feature, but in some cases it does not develop until after the acute abdominal episode. Most cases resolve satisfactorily with conservative management.     

恶性淋巴瘤继发的RS3PE综合征(附一例报告)

报告以缓解型血清阴性对称性滑膜炎伴凹陷性水肿(RS3PE)综合征为首发症状的恶性淋巴瘤1例，并复习文献。RS3PE综合征只是一种临床综合征，而非一个独立的疾病，有原发性与继发性之分。继发性RS3PE综合征常与肿瘤、风湿性疾病、血液病等密切相关。其预后与原发疾病密切相关。诊断和治疗时应注意查找其原发疾病。与肿瘤合并出现且随着肿瘤治疗缓解消失的RS3PE综合征称为副瘤性RS3PE综合征。当伴随全身症状与体征、血嗜酸细胞计数和(或)乳酸脱氢酶水平升高时应警惕恶性淋巴瘤。     

臀肌挛缩症概述

臀肌挛缩症(GMC)病因尚不明确,可由多种原因引起,主要累及臀肌肌纤维及筋膜纤维变性、挛缩而导致的临床综合征,以髋关节功能受限为临床表现,但其影像诊断报道较少.本文概述GMC的病因、临床表现、影像学诊断、鉴别诊断和手术方式,重点在于影像学检查在臀肌挛缩症中的应用价值,旨在为外科手术治疗提供帮助.     

嗜酸性细胞增多性皮炎21例分析

目的 探讨嗜酸性细胞增多性皮炎和高嗜酸性细胞增多综合症的临床特征、诊断、治疗及预后，以提高对嗜酸性细胞异常增多性皮炎的认识。方法 回顾性分析21例以皮肤、血液甚至骨髓嗜酸性细胞浸润为特征并伴有部分器官损害的病例。结果 这些病例是具有嗜酸性细胞增多性皮炎（HED），部分病例有非典型的器官损害但不能确诊为高嗜酸性细胞增多综合症（HES）的器官浸润的一组疾病。结论 这些伴有器官损害的HED可能是HES。建议对HED早期进行基因分析将有助于这些病例的治疗。     

Eosinophilic fasciitis in a 57-year-old Japanese-American woman

Eosinophilic fasciitis (EF) is a rare connective tissue disorder characterized by symmetrical sclerodermatous skin changes primarily affecting the extremities and histologically, by thickening of the fascia with chronic inflammatory infiltrate containing eosinophils. EF is associated with peripheral eosinophilia, hypergammaglobulinemia, and an elevated ESR. Reported is a case of EF in a 57-year-old Japanese-American woman who refused treatment with prednisone, review of other treatment options, and discussion of key differences between this disease and scleroderma.