格列卫治疗一例以咳嗽、发热为首发表现的特发性嗜酸粒细胞增多综合征及文献复习

目的 提高临床医师对特发性嗜酸粒细胞增多综合征的认识.方法 分析一例特发性嗜酸粒细胞增多综合征患者的临床资料及诊治经过,并复习相关文献.结果 患者,男,27岁,因“咳嗽伴畏寒发热1周,痰中带血1d”入院.多次复查血常规提示血嗜酸粒细胞增高,肺部CT提示双侧胸腔积液,心包积液,诊断为特发性嗜酸粒细胞增多综合征,给予甲基泼尼松龙治疗无效,后行血清FIP1L1/PDGFRα融合基因检测,结果提示阳性,改格列卫治疗,复查血常规提示血嗜酸粒细胞恢复正常.结论特发性嗜酸粒细胞增多综合征比较少见,临床表现不典型,故临床医师应提高对本病的认识.     

以肺栓塞为首发表现的高嗜酸粒细胞综合征一例并文献复习

目的 分析高嗜酸粒细胞综合征伴多发血栓形成的临床特征,提高临床对嗜酸粒细胞增多导致血栓性疾病的认识.方法 以“嗜酸粒细胞增高、血小板减少、深静脉血栓形成”为中文关键词,“hypereosinophilic,thrombocytopenia,deep vein thrombosis"为英文关键词检索2002年1月-2012年1月万方数据库和Pebmed数据库的相关文献,共获得9篇,其中中文5篇,英文4篇,排除未明确诊断的2篇文献,共纳入7篇,报道了8例患者,其中男5例,女3例,年龄24-75岁,结合文献报道的病例特点及本病例特征进行文献复习.结果 患者男,27岁,以胸痛气短伴痰中带血起病,经胸部强化CT确诊肺栓塞,同时有下肢深静脉血栓形成,实验室检查提示血嗜酸粒细胞增高和血小板下降.结合文献嗜酸粒细胞增高可损伤血管内皮细胞,导致多发动静脉血栓形成,抗凝同时应用糖皮质激素治疗有效.结论 高嗜酸粒细胞综合征是嗜酸粒细胞增多症的一种,临床以多器官受累为表现,伴多发血栓形成,抗凝治疗同时应用皮质激素可降低嗜酸粒细胞计数,减少其血管内皮损伤对血栓形成的影响.     

嗜酸粒细胞增多症53例临床分析

目的提高对嗜酸粒细胞增多症的认识。方法对北京协和医院1992-11~2005-02的嗜酸粒细胞增多症患者53例,予以回顾性分析。结果嗜酸粒细胞增多症的病因多种多样,反应性嗜酸粒细胞增多症中包括变态反应、感染、结缔组织疾病、肿瘤、嗜酸粒细胞性胃肠炎、呼吸系统疾病。特发性嗜酸粒细胞增多症18例表现为病程长、多系统累及,淋巴结肿大、脾大以及血栓的发生率高,白细胞、嗜酸粒细胞水平、免疫球蛋白(IgG)、C反应蛋白(CRP)水平较高。结论嗜酸粒细胞增多症的病因分布常广泛,临床表现各异,因此其诊断和鉴别诊断非常重要。     

特发性嗜酸粒细胞增多症1例

特发性嗜酸粒细胞增多症(idiopathic hypereosinophilic syndrome,IHES)亦称播散性嗜酸粒细胞增多症(disseminated eosinophilic disease).播散性嗜酸粒细胞胶原病(disseminated eosinophilic collage disease)是原因不明的嗜酸粒细胞持续增多(＞1.50×109)6个月以上,并伴有脏器损害.     

阿托伐他汀引起嗜酸粒细胞增多1例

<正>阿托伐他汀为常用降脂药物,该药引起嗜酸粒细胞增多罕见。本文报告1例阿托伐他汀致嗜酸粒细胞增多的病例,以引起临床医师对嗜酸粒细胞增多症病因的重视。临床资料患者男,60岁。因腹痛近2个月、嗜酸粒细胞增多2 d就诊于北京协和医院。2011年8月,患者被诊断为2型糖尿病、高脂血症、动脉粥样硬化、高血     

嗜酸粒细胞增多综合征合并脑梗死的临床特点研究

嗜酸粒细胞增多综合征是缺血性脑血管少见的病因,病因治疗是防止复发,改善预后的关键,临床医师应提高对本病的认识。回顾性分析3例嗜酸粒细胞增多综合征合并脑梗死患者的临床资料,旨在探讨嗜酸粒细胞增多综合征合并脑梗死的临床特点。     

特发性嗜酸粒细胞增多症、变应性肉芽肿性血管炎、非霍奇金淋巴瘤合并嗜酸粒细胞增多的临床特点比较

目的探讨特发性嗜酸粒细胞增多症(HES)、变应性肉芽肿性血管炎(CSS)、非霍奇金淋巴瘤(NHL)合并嗜酸粒细胞增多在临床表现、实验室检查、脏器受累等方面的异同,为三种疾病的鉴别诊断提供帮助。方法分析北京协和医院所有明确诊断为淋巴瘤、CSS的住院患者经查询病程存在慢性嗜酸粒细胞持续性升高者及所有明确诊断为HES的住院患者并加以比较,正态分布计量资料比较采用t检验,计量资料非正态和方差齐性条件,则采用非参数秩和检验,计数资料比较用χ2检验。结果 HES及CSS比较,嗜酸粒细胞绝对计数、IgE升高等均无统计学差异,而神经系统及肺部受累、淋巴结肿大、炎性指标升高等差异有统计学意义(P≤0.05)。CSS患者神经系统受累多见于外周神经,而HES多为中枢神经系统。CSS血管受累主要为血管炎,而HES多为动静脉血栓形成。HES与NHL合并嗜酸粒细胞增多比较,嗜酸粒细胞绝对值、淋巴结肿大、肺部受累、LDH升高均有统计学差异(均P≤0.05)。嗜酸粒细胞常见的浸润脏器如神经系统、胃肠道等在NHL合并嗜酸粒细胞增多病例中不多见。结论 HES、CSS、NHL合并嗜酸粒细胞增多三类疾病临床特点各有不同,诊断及鉴别需要通过仔细的脏器评估检查、实验室检查及密切的临床随诊。     

14例肺嗜酸细胞增多症误诊肺结核原因分析

目的通过对肺嗜酸细胞增多症误诊为肺结核的原因分析,提高对两种疾病的鉴别诊断能力.方法对14例经临床及实验室检查证实的肺嗜酸细胞增多症误诊肺结核的原因进行回顾性分析.结果误诊肺结核的肺嗜酸细胞增多症患者最常见的症状为咳嗽、咳黏痰、胸痛及低热、乏力、盗汗等全身中毒症状,病程常超过3周.胸部X线、CT表现多种多样.“Y”字形或葡萄状阴影,阻塞部位远端可有肺不张或阻塞性炎症.结论痰结核菌阴性而临床症状和X线表现与肺结核相似,经正规抗结核治疗后仍反复发作或无效的病人,应考虑到肺嗜酸细胞增多症.     

14例肺嗜酸细胞增多症误诊肺结核原因分析

目的通过对肺嗜酸细胞增多症误诊为肺结核的原因分析,提高对两种疾病的鉴别诊断能力。方法对14例经临床及实验室检查证实的肺嗜酸细胞增多症误诊肺结核的原因进行回顾性分析。结果误诊肺结核的肺嗜酸细胞增多症患者最常见的症状为咳嗽、咳黏痰、胸痛及低热、乏力、盗汗等全身中毒症状,病程常超过3周。胸部X线、CT表现多种多样。“Y”字形或葡萄状阴影,阻塞部位远端可有肺不张或阻塞性炎症。结论痰结核菌阴性而临床症状和X线表现与肺结核相似,经正规抗结核治疗后仍反复发作或无效的病人,应考虑到肺嗜酸细胞增多症。     

嗜酸粒细胞性胃肠炎临床分析24例

目的:分析嗜酸粒细胞性胃肠炎的临床特点、诊断要点、治疗及预后.方法:对本院确诊的24例嗜酸粒细胞性胃肠炎患者的病史、临床表现、实验室检查、内镜检查、病理检查、治疗及随访情况进行系统分析.结果:本组资料显示,有过敏史者占37.5%,过敏诱因者占25%.腹痛(87.5%)为最常见的临床表现.83.3%患者外周血嗜酸粒细胞计数升高,为(4.59±3.69)×109/L.内镜下主要表现为黏膜充血水肿、糜烂,以十二指肠最为明显,活检可证实大量嗜酸粒细胞浸润.腹水为渗出液,可见嗜酸粒细胞.激素治疗可在1 wk内迅速缓解症状,并使嗜酸粒细胞恢复正常.病情可有反复,但预后良好.结论:外周血嗜酸粒细胞计数、过敏史对诊断嗜酸粒细胞性胃肠炎有提示作用,内镜下黏膜活检和腹水嗜酸粒细胞增多是诊断的关键.糖皮质激素治疗优于饮食治疗,预后良好.     

Clonality of isolated eosinophils in the hypereosinophilic syndrome

The idiopathic hypereosinophilic syndrome (IHES) is a rare disorder characterized by unexplained, persistent eosinophilia associated with multiple organ dysfunction due to eosinophilic tissue infiltration. In the absence of karyotypic abnormalities, there is no specific test to detect clonal eosinophilia in IHES. Analysis of X-chromosome inactivation patterns can be used to determine whether proliferative disorders are clonal in origin. Methylation of HpaII and Hha I sites near the polymorphic trinucleotide repeat of the human androgen receptor gene (HUMARA) has been shown to correlate with X-inactivation. In this study, we have used the polymerase chain reaction (PCR) with nested primers to analyze X-inactivation patterns of the HUMARA loci in purified eosinophils from female patients with eosinophilia. Peripheral blood eosinophils were isolated by their autofluoresence using flow cytometric sorting. Eosinophils purified from a female patient presenting with IHES were found to show a clonal pattern of X-inactivation. Eosinophil-depleted leukocytes from this patient were polyclonal by HUMARA analysis, thus excluding skewedness of random X-inactivation. After corticosteroid suppression of her blood eosinophilia, a clonal population of eosinophils could no longer be detected in purified eosinophils. In contrast, eosinophils purified from a patient with Churg-Strauss syndrome and from six patients with reactive eosinophilias attributed to allergy, parasitic infection, or drug reaction showed a polyclonal pattern of X-inactivation by HUMARA analysis. The finding of clonal eosinophilia in a patient presenting with IHES indicates that such patients may have, in reality, a low-grade clonal disorder that can be distinguished from reactive eosinophilias by HUMARA analysis. Further, the method described can be used to monitor disease progression.     

Idiopathic hypereosinophilic syndrome-related pulmonary involvement diagnosed by bronchoalveolar lavage.

Patients with HES and pulmonary infiltrates may pose certain diagnostic problems as the infiltrates may be attributed to infection, infarction, congestive heart failure, or HES itself. We report an 87-year-old woman with idiopathic HES presenting with bibasal alveolar infiltrates. Differential cell count in BAL fluid yielded a very high percentage (73 percent) of eosinophils. Other authors previously mentioned the absence of eosinophils in the lavage fluid despite an important peripheral eosinophilia in a patient with the idiopathic HES but without HES-related pulmonary involvement. Thus, BAL fluid eosinophilia may suggest HES-related pulmonary involvement. Therefore, BAL might be an important diagnostic tool in the management of pulmonary infiltrates in idiopathic HES.     

The role of eosinophil morphology in distinguishing between reactive eosinophilia and eosinophilia as a feature of a myeloid neoplasm

Morphological features of eosinophils in patients with reactive eosinophilia (28 patients) and clonal eosinophilia (26 patients) have been compared with each other and with the eosinophil characteristics of healthy volunteers (three subjects) and of patients with the idiopathic hypereosinophilic syndrome (three patients). Morphological features, assessed in isolation from other haematological abnormalities, were found to have poor specificity for a myeloid neoplasm. The most useful feature was the presence of basophilic granules in mature eosinophils, which was associated particularly with acute myeloid leukaemia with inv(16). Marked reduction in granules occurred more often in some subsets of the myeloid neoplasm group but nevertheless was lacking in specificity since it was not infrequently seen in reactive eosinophilia. Although experienced morphologists more often considered that a myeloid neoplasm was likely in patients in whom this was the diagnosis (69%), myeloid neoplasia was also considered likely in a considerable proportion (39%) of patients with reactive eosinophilia. Morphological abnormalities of eosinophils therefore cannot be assessed in isolation in seeking to make a diagnosis of a myeloid neoplasm. Morphology is, however, needed and should be integrated with the results of other investigations.     

Diagnostic utility of pleural fluid eosinophilia

It has been stated that pleural fluid eosinophilia (defined as greater than 10 percent eosinophils in the pleural white cell differential count) is not helpful in the diagnosis of exudative effusions. By review of the recent literature, it was found that pleural fluid eosinophilia was associated most often with idiopathic effusions or with air previously introduced into the pleural space. Also, a high proportion of "idiopathic" and benign asbestos effusions were characterized by pleural fluid eosinophilia, a previously unrecognized phenomenon. The diagnostic utility of finding eosinophils in the pleural space was assessed from its impact on prior probabilities of disease. Estimates of pretest likelihoods of malignant versus nonmalignant pleural effusions and the prevalence of eosinophilia in effusions of known cause were obtained from extensive literature review. These were modified by using Bayes' rule to estimate the revised probability of disease in the presence of an eosinophilic effusion. The presence of pleural fluid eosinophilia considerably reduced the probability of malignancy or tuberculosis and increased the likelihood of an underlying benign disorder. Pleural fluid eosinophilia is a useful finding that can aid in the diagnosis of an exudative pleural effusion.     

Eosinophilic Disorders in Various Diseases

Peripheral and tissue eosinophilia are usually associated with a variety of inflammatory, malignant, and infectious conditions. As the presence of eosinophils in the tissues may cause significant cellular damage to vital organs such as the heart, tissue eosinophilia should be diagnosed and treated promptly. One operative way to evaluate eosinophilic disorders is to classify them into extrinsic and intrinsic. While extrinsic eosinophilic disorders are usually due to the production of eosinopoietic factors derived from T cells or tumor cells, the intrinsic types generally are the result of genetic mutations in the eosinophilic lineage. As we understand more the biology of eosinophils, only a few eosinophilic disorders remain idiopathic. The purpose of this article is to help the clinician classify in an operational manner most eosinophilic disorders, using the extrinsic and intrinsic model. This may facilitate not only a better understanding of the role of eosinophils in these disorders, but also help the systematic clinical work-up and potential treatment of affected patients.     

Churg-Strauss syndrome in a case of eosinophilia

A number of allergic, infectious and idiopathic diseases are associated with an increased number of eosinophils in blood. We report the case of a woman who was referred to our outpatient clinic due to asthma that had first developed three months previously and papular rash. Laboratory investigations revealed eosinophilia (23 %; 2,162 cells/ml). An allergic cause was ruled out by anamnesis, skin prick-test and specific IgE determination. Another frequent cause of eosinophilia is infestation by parasitic helminths, but serologic studies and studies of parasites in feces were negative. Chest radiography and computed tomography scan revealed diffuse infiltrates. The diagnosis was confirmed by transbronchial and skin lesion biopsies. The patient was finally diagnosed with Churg-Strauss syndrome and was treated with oral corticosteroids. A delay in the diagnosis and treatment of this syndrome increases the risk of death from the complications of vasculitis. Because of the importance of an early diagnosis in this disease, its presence should be suspected in cases of eosinophilia after ruling out more frequent causes.     

Eosinofilia sinovial

Synovial fluid eosinophilia is defined as the presence of eosinophils, irrespective of quantity, in the synovial fluid and is a rare finding that is probably underestimated. The pathogenesis of this entity remains incompletely understood. Secondary and idiopathic forms have been described. Idiopathic forms are those not associated with systemic or rheumatic inflammatory disease or associated with chronic non-inflammatory rheumatic diseases. Idiopathic forms can be divided into pure or pseudoallergic forms when they occur in patients with an atopic background and/or intensely positive dermographism. Both forms are usually monoarthritis of the large joints with a substantial component of joint effusion but few inflammatory signs. Synovial fluid usually contains between 2,000 and 10,000 leukocytes/mm³, with a variable percentage of eosinophils. Although a major form (>10% eosinophils) and a minor form (<10% eosinophils) have been distinguished, both seem to have the same significance in terms of clinical manifestations and prognosis. Peripheral eosinophilia (>600 eosinophils/mm³) is a rare association and is not usually severe. Symptoms resolve within a few days without specific therapy and recurrences occur in approximately half of patients. Non-steroidal anti-inflammatory drugs are usually sufficient to control symptoms. Synovial fluid eosinophilia has not been associated with the development of new joint deformities nor has it been described as a chronic form of arthritis.     

Interleukin 5 and granulocyte-macrophage colony-stimulating factor levels in bronchoalveolar lavage fluid in interstitial lung disease.

The purpose of this study was to evaluate the role of several eosinophil growth factors including interleukin (IL)-5, interleukin (IL)-3 and granulocyte-macrophage colony-stimulating factor (GM-CSF) in the pathogenesis of interstitial lung disease with eosinophilia. IL-5, IL-3 and GM-CSF in bronchoalveolar lavage fluid (BALF) were measured by enzyme-linked immunosorbent assay (ELISA) in patients with eosinophilic pneumonia (EP), bronchiolitis obliterans organizing pneumonia (BOOP), idiopathic pulmonary fibrosis (IPF), sarcoidosis and healthy volunteers. IL-5 in BALF was high only in patients with EP. IL-3 in BALF was undetectable in the majority of patients with these diseases. GM-CSF in BALF was detectable in 30-67% of each group of patients. In patients with BOOP and IPF, the number of eosinophils in BALF was higher in patients with detectable GM-CSF than in patients in whom GM-CSF was below the detection limit. Eosinophil cationic protein (ECP) was detected in all patients with EP and some with BOOP and IPF. There was a significant correlation between ECP levels and percentage or number of eosinophils in BALF. The results suggest the possibility that interleukin 5 in eosinophilic pneumonia, and granulocyte-macrophage colony-stimulating factor in bronchiolitis obliterans organizing pneumonia and idiopathic pulmonary fibrosis may play important roles in eosinophil recruitment in the lung. Activation of eosinophils in the lung is likely to be induced by both interleukin 5 and granulocyte-macrophage colony-stimulating factor.     

Pulmonary infiltrates with eosinophilia induced by nimesulide in an asthmatic patient

We report a case of pulmonary infiltrates with eosinophilia (PIE) induced by nimesulide in an asthmatic patient on regular inhaled corticosteroid treatment. A high level of eosinophils in induced sputum was detected. No evidence of bacterial or fungal infection was observed. The patient responded rapidly to corticosteroid treatment. Rechallenge with nimesulide induced blood and sputum eosinophilia, confirming our diagnosis. Nimesulide, though it is a relatively safe drug to use in aspirin-sensitive asthmatics, may (like other nonsteroidal anti-inflammatory drugs) cause PIE in asthmatic patients and thus should be used cautiously.     

Clinical and inflammatory features of asthma with dissociation between fractional exhaled nitric oxide and eosinophils in induced sputum

Background: Measurement of the fractional exhaled nitric oxide (FeNO) and eosinophils in induced sputum are noninvasive markers for assessing airway inflammation in asthma. The clinical usefulness of the correlation between raised FeNO and sputum eosinophilia is controversial. We aimed to examine dissociation between FeNO and sputum eosinophils in a clinical series of asthma patients and to determine whether dissociation between these noninvasive markers was associated with clinical and inflammatory differences in these patients. Methods and findings: A total of 110 patients with asthma were included in a cross-sectional study. All of them were on maintenance treatment for asthma. All patients underwent the following on the same day: FeNO, induced sputum, spirometry, serum total IgE levels and skin prick test. The level of asthma control was determined by the Asthma control Test Questionnaire. In 46 (41.8%) patients, a discrepancy between FeNO and sputum eosinophil count was observed, of those, 34 (73.9%) had a FeNO <50?ppb and high eosinophil count, and were characterized by having a predominance of nonallergic asthma with bronchial eosinophilic inflammatory phenotype. Also, 12 (26.1%) patients had FeNO ≥50?ppb and sputum eosinophilia within the normal reference values, and were characterized by having a predominance of atopy with a paucigranulocytic inflammatory phenotype. Conclusions: A high percentage of patients with dissociation between results of FeNO and sputum eosinophils was observed. These patients showed differential clinical and inflammatory features.