嗜酸粒细胞性肺病:亟待澄清认识

嗜酸粒细胞性肺病(eosinophilic lung diseases,ELD)是指以气道和(或)肺实质嗜酸粒细胞增多为特征的一组异质性临床疾病,伴有或不伴有外周血嗜酸粒细胞增多[1].以前与ELD相关的名称较多,如嗜酸粒细胞肺浸润、肺嗜酸粒细胞增多症、嗜酸粒细胞性肺炎和肺嗜酸粒细胞综合征等,但目前多以ELD代替[2-5].     

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     

肺嗜酸粒细胞增多症误诊为肺结核一例

嗜酸粒细胞增多症是一组多病因引起的疾病,发病多与变态反应或异常的免疫反应有关,变态原可能是感染、外界吸入或自身免疫性的。本文报告1例老年男性嗜酸粒细胞增多症误诊为肺结核的案例进行分析。     

胸水嗜酸粒细胞增多症

胸水嗜酸粒细胞增多症 (pleural fluid eosinophilia,PFE,也称嗜酸性胸水 )不是独立的疾病 ,而是一组伴有胸水白细胞分类嗜酸粒细胞超过 10 %的临床综合征 ,可伴有或不伴有血嗜酸粒细胞增多 ,病因不同而临床表现、预后各异。虽然其不能作为一个独立的诊断 ,但它的出现能缩小渗出性胸腔积液的鉴别诊断范围。1　流行病学自从 Harmsen于 1894年报告了第 1例 PFE后 ,该病越来越受到临床医师的重视。其发生率约占渗出性胸水的 6 .0 - 12 .6 %。 Adelman[1 ] 总结了 34 3例 PFE,发现有 74%是由特发性胸腔积液、气胸及胸腔感染引起的 (见表 1)…     

嗜酸性粒细胞增多症并发多发性脑梗死一例并文献复习

作者回顾性分析了1例合并急性多发性脑梗死的嗜酸粒细胞增多症患者的临床资料。患者青年男性,因颈痛、四肢乏力入诊广州医科大学附属第六医院,患者既往有食物过敏史1年。患者入院时存在嗜酸性粒细胞增多,头部MRI示急性多发性脑梗死。给予糖皮质激素治疗后,症状迅速好转。嗜酸粒细胞增多症是脑梗死的少见病因。当急性多发性脑梗死患者出现嗜酸性粒细胞增多,在排除了其他导致脑梗死的病因后,需警惕嗜酸粒细胞增多症导致脑梗死可能。嗜酸粒细胞增多症合并脑梗死的患者建议糖皮质激素治疗。     

原因不明的嗜酸粒细胞增多症36例分析

目的总结原因不明的嗜酸粒细胞增多症的临床表现、实验室检查及诊治方法。方法结合文献复习,对36例原因不明的嗜酸粒细胞增多症患者的临床资料进行分析。结果原因不明的嗜酸粒细胞增多症患者的临床及实验室检查均有别于其他有原因的嗜酸粒细胞增多症;原因不明的嗜酸粒细胞增高可能为原发性嗜酸粒细胞增多症(HES)的早期病变。结论建议对嗜酸粒细胞明显增高的原因不明的嗜酸粒细胞增多症患者,应积极治疗,防止多器官损害,从本临床分析及文献看,强的松应是本病首选而有效的药物。     

以支气管哮喘为首发症状的嗜酸粒细胞性肺浸润症1例并文献复习

目的 探讨单纯性肺嗜酸粒细胞增多症的临床特征、诊断及治疗.方法 分析就诊于上海市普陀区人民医院呼吸科的1例以支气管哮喘(简称哮喘)为首发症状的单纯性肺嗜酸粒细胞增多症患者的临床资料,并结合文献进行复习.结果 单纯性肺嗜酸粒细胞增多症为少见疾病,主要以咳嗽为主,影像学上缺乏特异性,多表现为游走性浸润影.明确诊断主要依靠病史、诱导痰或支气管肺泡灌洗检测.治疗以应用糖皮质激素为主.结论 单纯性肺嗜酸粒细胞增多症是一组以外周血嗜酸粒细胞增多、胸部X线呈短暂游走性浸润影、轻微全身和呼吸系统症状为特征的综合征,因临床症状不典型,有时可能会出现类似哮喘的表现.所以,需要临床综合分析做出判断.     

重视嗜酸粒细胞增多对神经系统疾病的诊断价值

嗜酸粒细胞增多综合征(hypereosinophilic syndrome)这一概念最初于1968年提出,定义为一组具有类似临床特征的患者群,主要表现为慢性外周血嗜酸粒细胞增多和嗜酸粒细胞浸润导致的器官损伤.1975年Chusid基于临床实践经验进一步建立了嗜酸粒细胞增多综合征的临床诊断标准.随着分子生物学和遗传学的研究进展,部分嗜酸粒细胞增多综合征已找到相应病因.近年来在神经科临床工作中,我们发现神经系统疾病与嗜酸性粒细胞增多有一定的关联,希望临床医生能加以重视.     

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.     

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。     

Recurrent angioedema and hypereosinophilia

IntroductionAmong the various causes of eosinophilia are the syndrome first described by Gleich in 1984. This syndrome is characterized by angioedema of the face, neck, extremities and trunk, weight gain, hypereosinophilia (60-70 %), fever, and increased serum IgM levels without involvement of the vital organs.Case reportA 17-year-old non-allergic woman was referred to our hospital for further investigation of recurrent angioedema, initially of the hands and feet and subsequently of the face, with onset 3 years previously. The attacks had become more frequent and severe and had occurred monthly in the previous year. The patient also showed general malaise, without fever. Complementary investigations revealed eosinophils 40.8 %, total count 3,300/mm³, and serum IgM levels 343 mg/dl (normal range: 53-300 mg/dl). Possible causes of hypereosinophilia and eosinophilic infiltration of vital organs were ruled out. The patient was treated with oral corticosteroids which produced clinical remission and reduction of eosinophil count (1.7 %, total 200/mm³).DiscussionGleich syndrome is uncommon and has well-defined clinical features and a benign course. We describe a patient who presented the clinical characteristics of this syndrome with good response to steroids and without involvement of vital organs.ConclusionsOur patient presented clinical features compatible with a diagnosis of Gleich syndrome. Other entities associated with hypereosinophilia were ruled out.     

World Health Organization-defined eosinophilic disorders: 2011 update on diagnosis, risk stratification, and management

DISEASE OVERVIEW: The eosinophilias encompass a broad range of non-hematologic (secondary or reactive) and hematologic (primary, clonal) disorders with potential for end-organ damage. DIAGNOSIS: Hypereosinophilia has generally been defined as a peripheral blood eosinophil count greater than 1,500/mm(3) and may be associated with tissue damage. After exclusion of secondary causes of eosinophilia, diagnostic evaluation of primary eosinophilias relies on a combination of morphologic review of the blood and marrow, standard cytogenetics, fluorescent in situ-hybridization, flow immunocytometry, and T-cell clonality assessment to detect histopathologic or clonal evidence for an acute or chronic myeloid or lymphoproliferative disorder. RISK STRATIFICATION: Disease prognosis relies on identifying the subtype of eosinophilia. After evaluation of secondary causes of eosinophilia, the 2008 World Health Organization establishes a semi-molecular classification scheme of disease subtypes including myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB, or FGFR1, chronic eosinophilic leukemia, not otherwise specified (CEL, NOS), lymphocyte-variant hypereosinophilia, and idiopathic hypereosinophilic syndrome (HES), which is a diagnosis of exclusion. RISK-ADAPTED THERAPY: The goal of therapy is to mitigate eosinophil-mediated organ damage. For patients with milder forms of eosinophilia (e.g. < 1,500/mm(3) ) without symptoms or signs of organ involvement, a watch and wait approach with close-follow-up may be undertaken. Identification of rearranged PDGFRA or PDGFRB is critical because of the exquisite responsiveness of these diseases to imatinib. Corticosteroids are first-line therapy for patients with lymphocyte-variant hypereosinophilia and HES. Hydroxyurea and interferon-alpha have demonstrated efficacy as initial treatment and steroid-refractory cases of HES. In addition to hydroxyurea, second line cytotoxic chemotherapy agents and hematopoietic cell transplant have been used for aggressive forms of HES and CEL with outcomes reported for limited numbers of patients. Although clinical trials have been performed with anti IL-5 (mepolizumab) and anti-CD52 (alemtuzumab) antibodies, their therapeutic niche in primary eosinophilic diseases and HES have yet to be established.     

Hypereosinophilia associated with increased serum levels of carcinoembryonic antigen

We report a case of hypereosinophilia associated with increased serum levels of carcinoembryonic antigen (CEA). The patient developed fever, diarrhea, erythroderma and eosinophilia. Disorders known to be associated with eosinophilia were not detected. The typical malignant diseases related to a rise in CEA levels were not identified. The CEA value reached a maximum of 81.4 ng/ml a few weeks late for the peak of the eosinophilic count. Corticosteroid therapy was effective in improving clinical symptoms and the CEA values decreased in association with the improvement of those manifestations, suggesting a pathophysiological link between the disease activity of hypereosinophilia and the changes in CEA level.     

Eosinophilia: A study of 100 hospitalized patients

BACKGROUND: Eosinophilia accompanies a large number of diseases and conditions, but information is lacking about the clinical characteristics of patients who are hospitalized due to this abnormality. Our aim was to determine the clinical profile and most informative diagnostic tests in patients hospitalized in a tertiary hospital because of hypereosinophilia. METHODS: A retrospective review was done of the medical records of all patients hospitalized in a large urban medical center due to hypereosinophilia. All relevant clinical, laboratory, and imaging data were analyzed. RESULTS: A total of 100 patients were included in the study (58 males and 42 females, mean age 55.2+/-29 years). The blood eosinophil blood count was 4107+/-7254/mul (mean+/-SD) and this usually persisted for a few months. The cause of the eosinophilia was asthma or other atopic disease in 13% of the cases, allergic drug reaction in 6%, eosinophilic pneumonia in 10%, neoplastic diseases in 10%, idiopathic hypereosinophilic syndrome in 8%, Churg-Strauss Syndrome in 4%, infections in 10%, allergic fungal disease in 2%, and skin diseases in 3%; the cause remained unknown in 34% of cases. CONCLUSION: Guidelines are suggested for the investigation of patients with eosinophilia, including the level of the eosinophilia associated with specific diseases and the most informative diagnostic tests.     

Malignancy: Case Report: Hypereosinophilia Progressing to Granulocytic Sarcoma and Acute Myelocytic Leukemia with Trisomy 8: A Case Report and Review of the Literature

Conditions associated with increased peripheral blood and bone marrow eosinophil count may be reactive, clonal or idiopathic. Clonal eosinophilic disorders are characterized by increased production of eosinophils alongside a clone of malignant cells. In these patients, the eosinophils can either be demonstrated as being part of the malignant clone or produced as a result of cytokine production by the malignant clone. Criteria for the diagnosis of idiopathic hypereosinophilic syndrome (HES) include the exclusion of other known causes of hypereosinophilia. A few patients with the initial diagnosis of HES develop clonal disorders manifested by granulocytic sarcoma or acute leukemia. We report a patient with a nine year history of HES before progressing to chloroma and acute leukemia. Cytogenetic studies on the bone marrow specimen revealed trisomy 8. This report and others in the literature support the concept that at least some cases of HES are as yet unidentified clonal diseases. Cytogenetic studies are therefore recommended at diagnosis and during the follow up of patients with HES.     

Natural history of severe eosinophilia with uncertain aetiology and proposals on a practical approach to its management

Background: Eosinophilia is commonly encountered during clinical practice. Some can be attributed to well‐defined causes while others cannot. Optimal management of hypereosinophilia with unknown aetiology is uncertain as the natural history is not well described. Methods: We retrospectively studied patients with hypereosinophilia (>5 × 10⁹/L) and described the characteristics, natural history and treatment of those with eosinophilia of uncertain aetiology. Results: There were 141 patients with hypereosinophilia: 87 with well‐defined causes, 54 with uncertain aetiology. The latter was managed as hypereosinophilic syndrome (HES) (n= 5), idiopathic hypereosinophilia (IH) (n= 11), presumptive helminthic infection (n= 11) and reactive eosinophilia (n= 5), while 22 were insufficiently investigated and did not have definite working diagnoses. Their median age and peak eosinophil count were 64 (22 to 94) years and 10.0 (5.2–33.9) × 10⁹/L respectively. Forty‐six per cent had symptoms attributable to eosinophilia, with the HES and insufficiently investigated groups having the highest (100%) and lowest (27%) percentages respectively. HES and IH patients were most extensively investigated. All 14 HES or IH patients who received steroids responded. All presumptive helminthic infection patients received mebendazole: nine responded, and two had unassessable responses. For the remaining patients, seven received steroids and all responded; one received mebendazole but defaulted; 19 were not treated:11 resolved spontaneously. No non‐HES patients developed eosinophilia‐related organ dysfunction. No mortality was caused by hypereosinophilia. Conclusions: Patients with hypereosinophilia of uncertain aetiology can be empirically managed according to working diagnoses derived from history taking, examination and selective investigations. Most patients have benign short‐term outcomes, but longer monitoring is required to assess long‐term outcomes from untreated hypereosinophilia.     

Lymph-node-based malignant lymphoma and reactive lymphadenopathy in eosinophilic fasciitis.

BACKGROUND: Lymph node enlargement in patients with eosinophilic fasciitis is a rare occurrence and its clinical significance is unknown. METHODS: The literature and authors' registries were searched for eosinophilic fasciitis associated with lymphadenopathy. Clinical data, time sequence of appearance of either disorder, and pathological diagnoses were analyzed. RESULTS: Six patients presenting with eosinophilic fasciitis had a lymph-node-based lymphoma and 4 patients had a reactive lymphadenopathy. The patients with lymphoma were elderly and the subcutaneous induration preceded the lymphadenopathy by 2 to 36 months. The patients with eosinophilic fasciitis and reactive lymphadenopathy were young and the onset of subcutaneous induration and lymph node enlargement coincided with one another. Favorable response of the eosinophilic fasciitis to prednisone therapy was attained in 3 of 3 patients with reactive lymphadenopathy and in 4 of the 6 cases with lymphoma. CONCLUSIONS: Eosinophilic fasciitis is rarely associated with clinically significant lymph node enlargement. Subcutaneous induration preceding the lymphadenopathy by 6 months or more, especially in elderly patients, suggests an underlying lymphoma. A favorable response of the subcutaneous induration to prednisone treatment does not exclude the diagnosis of lymphoma; therefore, it does not supersede the need of a pathological evaluation. A lymph node biopsy is mandatory in all cases.     

Two cases of eosinophilic vasculitis with thrombosis

Vasculitides are characterized by vessel wall inflammation of unknown etiology. We report two cases of eosinophilic vasculitis and hypereosinophilia with thrombosis. They have been treated with a high-dose glucocorticoid and anticoagulation. These cases emphasize that thrombosis should be anticipated in patients with eosinophilic vasculitis.     

The pathogenesis of eosinophilic endomyocardial disease in patients with carcinomas of the lung

Summary Studies were done on a patient with a carcinoma of the lung induced by hypereosinophilia who was thought to be at risk from developing eosinophilic endomyocardial disease to see whether the development of heart disease could be related to abnormalities in the morphology or kinetics of blood eosinophils. The patient was a 61-year-old man who had a partial resection of a squamous cell bronchial carcinoma of anaplastic large cell type which had spread locally. Seven months later, he developed a blood eosinophil count of 33.9 × 10⁹/l. There were only transient responses to treatment with steroids and tumor irradiation, and he died 15 weeks later. Up to 3 × 10⁹/l blood eosinophils were degranulated, correlating with serum levels of eosinophil cationic protein. The blood half-life of¹¹¹indium-labeled eosinophils was prolonged to 53 h, but their distribution was normal. Although an unsuccessful search was made during life for the development of endomyocardial damage, at postmortem the left ventricle had features of eosinophilic endomyocardial disease in the acute necrotic stage. Among 13 other reported patients with carcinoma of the lung and hypereosinophilia, three also had endomyocardial disease or myocardial lesions. These findings confirm the suggestion that the presence in the blood of >1×10⁹/l degranulated eosinophils can be used to predict the development of eosinophilic endomyocardial disease before it becomes apparent clinically, and they also add weight to the hypothesis that blood eosinophil degranulation causes this complication of hypereosinophilic states.