儿童嗜酸性粒细胞增多症27例

目的:总结嗜酸性粒细胞增多症(eosinophilia)的病因、临床表现及实验室资料,加深对本病的认识。方法:对1995年1月至2005年7月广州市花都区人民医院儿科收住院的27例嗜酸性粒细胞增多症患儿的病因、临床表现、实验室检查、治疗等进行回顾性分析。结果:嗜酸性粒细胞增多症病因主要是寄生虫感染(11例)、变态反应性疾病(8例)、原因不明(8例),11例肠道寄生虫感染有8例为肠道蛔虫症。临床表现为发热(37.0%)、咳嗽(29.6%)、皮疹(18.5%)、消化系统症状(25.9%),经治疗预后良好。结论:嗜酸性粒细胞增多症主要病因是寄生虫感染及变态反应性疾病,寄生虫感染以蛔虫感染为主。近年来寄生虫感染所致嗜酸性粒细胞增多症有下降趋势,而变态反应性疾病引起嗜酸性粒细胞增多症有上升趋势。     

嗜酸性肺部疾病分类及诊断方法再认识

<正>嗜酸性肺部疾病(ELD)是一组在血液、呼吸道、肺泡和(或)组织间隙中嗜酸性粒细胞升高为临床特征的疾病[1]。ELD在1936年最先被命名为Lffler综合征。1943年Weingarten用热带嗜酸性粒细胞增多症(TPE)来描述一组具有痉挛性支气管炎、外周血嗜酸性粒细胞增多、双肺弥漫性斑片阴影的综合征。1952年Reeder与Goodrich将之命名为肺嗜酸性粒细胞浸润症,     

中国嗜酸性粒细胞增多症诊断和治疗指南(2024版)

嗜酸性粒细胞增多症包括一组非血液系统(继发或反应性)和血液系统(原发或克隆性)疾病, 该组疾病可能伴有器官受损。基于新的临床研究数据和对疾病分子遗传学解析结果, 2022年世界卫生组织(WHO)和国际共识分型(ICC)对嗜酸性粒细胞增多性疾病的诊断和分型标准进行了更新。本指南是在《嗜酸性粒细胞增多症诊断和治疗中国专家共识(2017年版)》基础上的更新, 旨在为我国血液学工作者提供一个规范的嗜酸性粒细胞增多症的诊断程序、实验室检查、诊断标准和治疗原则。     

以消化道症状为主要表现的嗜酸性粒细胞增多症14例

目的：提高对以消化道症状为主的嗜酸性粒细胞增多症的认识。方法：对14例以消化道症状为主要表现的嗜酸性粒细胞增多症患者的临床资料进行回顾性分析。结果：寄生虫感染6例，嗜酸粒细胞胃肠炎3例，溃疡性结肠炎2例。结核性腹膜炎2例，恶性腹水1例。结论：对以消化道症状为主要表现、原因不明的嗜酸性粒细胞增多症．应考虑一些少见病因，并及时进行必要的检查，避免误诊误治。     

嗜酸性粒细胞增多症18例临床分析

目的探讨嗜酸性粒细胞增多症的病因和临床治疗效果。方法回顾分析2000年5月至2009年11月住院的18例嗜酸性粒细胞增多症的临床资料。结果病因主要为结缔组织病6例、过敏性疾病6例、感染性疾病2例、原因不明的特发性嗜酸性粒细胞增多综合征（IHES）2例。除恶性肿瘤2例外,经治疗其他16例均预后良好。结论继发性嗜酸性粒细胞增多症患者治疗,首先应给予病因治疗,必要时辅以糖皮质激素对症治疗,常能收到较满意疗效;对于原因不明的特发性嗜酸性粒细胞增多综合征（IHES）并发多脏器功能损害患者治疗,应给予糖皮质激素和酪氨酸激酶抑制剂伊马替尼（中国商品名：格列卫）联合治疗,可取得良好效果。     

1例嗜酸粒细胞增多症并发脑梗死病人的护理

<正>嗜酸粒细胞增多综合征是一组嗜酸粒细胞持续升高,并伴有器官损害的疾病,最早在1968年由Hardy报道[1],由于其病因不明,故又称为特发性嗜酸粒细胞增多综合征(idiopathic hypereosinophilic syndrome,HES)。成人外周血嗜酸性粒细胞分类计数0.07,绝对值0.45×109/L时称为嗜酸性粒细胞增多症。当外周血中嗜酸性粒细胞计数绝对值持续1.5×109/     

不洁饮食引起嗜酸粒细胞增多症15例分析

目的 分析因不洁饮食引起的爆发性嗜酸性粒细胞增多症15例,提高对以消化道症状为主的继发性嗜酸性粒细胞增多症的认识.方法 对15例爆发性嗜酸性粒细胞增多症患者的临床资料进行回顾性分析.结果 15例患者均在同一时间食用含有淋巴腺组织的不洁猪肉,陆续出现以恶心、呕吐、腹泻、腹胀等腹部不适症状,便培养没有检测出病原菌及寄生虫.白细胞计数(10.4～56.8)×109/L,嗜酸性粒细胞比例为0.165～0.682,绝对值(0.6～20.6)×109/L;骨髓检查8例,骨髓中嗜酸性粒细胞占0.232～0.565,以成熟细胞为主;骨髓细胞染色体核型分析为正常核型;FIP1 L1-PDGFRA融合基因阴性.1例轻症患者经对症治疗临床症状缓解,14例中至重症患者均给予激素为主的治疗法方案好转,但其中3例患者治疗2月后自行停药导致复发,再次给予激素为主的治疗有效.结论 对因不洁饮食引起的嗜酸性粒细胞增多症,应及时进行必要的检查以便与原发性嗜酸性粒细胞增多症鉴别,重症者需要采取激素为主的治疗方案,治疗应充分,疗程应足够长.     

嗜酸性粒细胞增多症的细胞遗传学研究

为探讨染色体异常克隆在嗜酸性粒细胞增多症诊断和鉴别诊断中的意义及克隆性嗜酸性粒细胞增多症涉及的染色体异常,收集了65例嗜酸性粒细胞增多患者的骨髓标本,培养24小时,采用G显带进行核型分析。结果表明：65例中9例拟诊为急性髓细胞性白血病-M4Eo检出特异性的染色体异常inv（16）,而其余的56例以嗜酸性粒细胞增多待诊的患者中5例检出染色体异常克隆,检出率为8.9%。根据临床、血液学资料并结合染色体检出结果,5例患者最后分别被诊断为急性髓系白血病伴嗜酸性粒细胞增多、慢性嗜酸性粒细胞白血病、8p11骨髓增殖综合征、慢性髓系白血病急变、急性髓系白血病-M4Eo。检出的染色体异常克隆分别为＋14、t（5;12）（q31;p13）、t（8;9）（p11;q32）、t（9;22）（q34;q11）和inv（16）（p13q22）。结论：在嗜酸性粒细胞增多症的诊断中,染色体的检测是判定克隆性和诊断慢性嗜酸性粒细胞白血病的重要手段,应作为常规的检测。     

嗜酸性粒细胞增多症的细胞遗传学研究

为探讨染色体异常克隆在嗜酸性粒细胞增多症诊断和鉴别诊断中的意义及克隆性嗜酸性粒细胞增多症涉及的染色体异常，收集了65例嗜酸性粒细胞增多患者的骨髓标本，培养24小时，采用G显带进行核型分析。结果表明：65例中9例拟诊为急性髓细胞性白血病-M4Eo检出特异性的染色体异常inv（16），而其余的56例以嗜酸性粒细胞增多待诊的患者中5例检出染色体异常克隆，检出率为8．9％。根据临床、血液学资料并结合染色体检出结果，5例患者最后分别被诊断为急性髓系白血病伴嗜酸性粒细胞增多、慢性嗜酸性粒细胞白血病、8p11骨髓增殖综合征、慢性髓系白血病急变、急性髓系白血病-M4Eo。检出的染色体异常克隆分别为＋14、t（5；12）（q31；p13）、t（8；9）（p11；q32）、t（9；22）（q34；q11）和inv（16）（p13q22）。结论：在嗜酸性粒细胞增多症的诊断中，染色体的检测是判定克隆性和诊断慢性嗜酸性粒细胞白血病的重要手段，应作为常规的检测。     

云南省德宏州农村地区人群嗜酸性粒细胞增多的调查

目的:探讨云南省德宏州农村地区人群嗜酸性粒细胞增多的可能原因以及德宏州地区5个少数民族之间嗜酸性粒细胞水平和地区间分布的差异。方法:采用2007年至2008年行地中海贫血基因调查所收集3333名研究对象的血标本,统计分析嗜酸性粒细胞检测结果并绘制频数分布图。结果:在德宏州农村地区,嗜酸性粒细胞增多现象较明显,占受调查人群的45.37%,且平均水平较高,平均值为0.63×109/L,其白细胞分类数为0.0705,高于全国参考值水平(P<0.05)。各民族间嗜酸性粒细胞增多的情况有差异,德昂族、傈僳族人群嗜酸性粒细胞增多的百分比较汉族和傣族人群明显(P均<0.05);各年龄组之间也有差异,随着年龄增长,嗜酸性粒细胞计数有增高趋势;各地区之间嗜酸性粒细胞水平的差异不显著。结论:云南省德宏州农村地区人群有嗜酸性粒细胞增多的现象,不同民族、各年龄段之间的嗜酸性粒细胞增多人数的百分比有差异;该人群嗜酸性粒细胞轻度增多最主要的原因推测为寄生虫感染所致。     

氟达拉宾治疗慢性淋巴细胞白血病诱发嗜酸细胞增多症1例报告并文献复习

目的:探讨氟达拉宾治疗慢性淋巴细胞白血病(CLL)诱发嗜酸细胞增多症的临床特点、诊断及治疗方法,减少误诊误治的发生。方法:总结1例诊断及治疗经过,结合文献对临床特点进行复习。结果:此病多发生在用药后2.4个疗程,大多无症状,少数患者发生皮损,皮损重者可应用激素及抗组胺药治疗。结论:氟达拉宾及2-氯脱氧腺苷(2-CdA)治疗淋巴增殖性疾病可诱发嗜酸细胞增多症,用药期间监测血象有助于早期诊断。     

Blood eosinophilia: a new paradigm in disease classification, diagnosis, and treatment

Acquired blood eosinophilia is considered either a primary or a secondary phenomenon. Causes of secondary (ie, reactive) eosinophilia include tissue-invasive parasitosis, allergic or inflammatory conditions, and malignancies in which eosinophils are not considered part of the neoplastic process. Primary eosinophilia is classified operationally into 2 categories: clonal and idiopathic. Clonal eosinophilia stipulates the presence of either cytogenetic evidence or bone marrow histological evidence of an otherwise classified hematologic malignancy such as acute leukemia or a chronic myeloid disorder. Idiopathic eosinophilia is a diagnosis of exclusion (ie, not secondary or clonal). Hypereosinophilic syndrome is a subcategory of idiopathic eosinophilia; diagnosis requires documentation of both sustained eosinophilia (absolute eosinophil count > or = 1500 cells/microL for at least 6 months) and target organ damage (eg, involvement of the heart, lung, skin, or nerve tissue). Genetic mutations involving the platelet-derived growth factor receptor genes (PDGFR-alpha and PDGFR-beta) have been pathogenetically linked to clonal eosinophilia, and their presence predicts treatment response to imatinib. Accordingly, cytogenetic and/or molecular investigations for the presence of an imatinib-sensitive molecular target should accompany current evaluation for primary eosinophilia. In the absence of such a drug target, specific treatment is dictated by the underlying hematologic malignancy in cases of clonal eosinophilia; however, the initial treatment of choice for symptomatic patients with hypereosinophilic syndrome is prednisone and/or interferon alfa.     

变应性肉芽肿性血管炎11例临床分析

【目的】分析变应性肉茅肿性血管炎(CSS)的临床表现．以便提高临床诊断水平【方法】11例确诊CSS患者．分析其既往痛史、临床表现、实验室检查、活检病理及治疗情况【结果】11例患者中发生哮喘10例;10例出现皮肤损伤;分别有1例和2例出现周围神经和中枢神经病变：消化道症状者1例．包括腹痛、腹泻和脓血便：肾脏损害者4例;心脏受累者6例全部病例周围血嗜酸粒细胞(EOS)均增高．有1例活捡病理支持CSS诊断。所有患者均用糖皮质激素和免疫抑制制治疗。【结论】CSS的临床表现多种多样．对于有系统性表现．特别是血嗜酸细胞升高者应提高警惕。     

Autochthonous strongyloidosis in an 81-year-old woman]

Strongyloidosis is an parasitic disease, caused by an intestinal nematode endemic in tropic and subtropic regions. In Central Europe it occurs only sporadically. The infective larvae in the soil penetrate the human skin. Following circulation through the lungs the larvae settle in the small intestine and mature into adult worms. Chronic strongyloidosis recurring up to 15 years is possible through endogenous autoinfection. Clinical feature of the disease are gastrointestinal symptoms, hypereosinophilia and skin rashes. We describe the case of an 81-year-old woman who presented with scaly exanthema, fever and perianal fistulation. A microscopic examination of a stool sample demonstrated filariform larvae of Strongyloides stercoralis. An autochthonous mode of infection was assumed. After starting treatment with mebendazole eosinophilia and rash gradually disappeared. The laboratory finding of eosinophilia in patients with gastrointestinal symptoms or exanthema should prompt the differential diagnosis of a parasitosis. Stool examination is necessary to find rare autochthonous infections by intestinal nematodes. Pathogenesis, clinical manifestation and treatment of strongyloidosis are discussed along with the clinical picture.     

Diagnosis of allergic bronchopulmonary aspergillosis

Five patients (4 men and 1 woman) afflicted with bronchopulmonary aspergillosis (ABPA) were examined. The main criteria for the diagnosis were a high level of the total IgE, demonstration of specific IgE to Aspergillus fumigatus, positive skin tests with antigens of moldy fungi. All the 4 patients with active process, not given corticosteroids, manifested eosinophilia of the sputum and peripheral blood. The clinical picture of bronchial asthma was recorded in 2 patients. It is desirable that all the patients with protracted and relapsing infiltrative processes in the lungs running with sputum and blood eosinophilia undergo examinations for the presence of ABPA.     

Idiopathic hypereosinophilic syndrome: a rare but fatal condition presenting with common symptoms

INTRODUCTION: Idiopathic hypereosinophilic syndrome (IHES) is a leukoproliferative disorder characterized by cytokine-induced overproduction of eosinophils with resultant multiorgan infiltration and damage. The diagnostic criteria includes evidence of end organ damage, exclusion of all other causes of eosinophilia and sustained absolute eosinophil count (AEC) > 1,500 cells/mcl for at least 6 months. CASE: An 88-year-old Caucasian female presented with persistent severe chronic cough, weight loss and rhinorrhea unresponsive to various treatments. Her workup during admission revealed absolute eosinophil count of 17,447 and bone marrow biopsy showed eosinophilia with no lymphoproliferative process. After excluding all other causes of eosinophilia, a diagnosis of IHES was made and prednisone was started. Symptoms resolved and her absolute eosinophil count progressively decreased. CONCLUSION: IHES mimics several other diseases. Mild eosinophilia should be worked up irrespective of age and sex. A high index of suspicion is required for earlier diagnosis and treatment, which could reduce morbidity and mortality.     

The idiopathic hypereosinophilic syndrome.

Idiopathic hypereosinophilic syndrome (HES) is empirically defined as the presence of prolonged eosinophilia without an identifiable underlying cause, and with evidence of end-organ dysfunction. Virtually any organ system may be involved, most frequently the heart, the central and peripheral nervous system, the lungs and the skin. We report on two clinical cases where the diagnosis of HES, with all the conventional criteria met, was proposed. In the first patient with HES, cardiac and pulmonary involvement was present. Skin changes and lung involvement were observed in the second reported patient with HES. In both patients there was prompt improvement of all clinical signs and symptoms of HES soon after treatment with methylprednisolone was begun. In the first patient long-term methylprednisolone therapy was healed successfully. For 2 years he has shown no clinical signs or symptoms of HES. The second patient is still undergoing long-term therapy with 4 mg of methylprednisolone daily. The histologic findings of the skin biopsy in the second patient were not typical for HES, but skin changes completely healed after corticosteroid therapy. This could mean that that the described skin changes were one of the HES skin manifestations. The other possibility is that the skin changes emerge in coincidence with HES.     

Drug rash with eosinophilia and systemic symptoms (DRESS) probably induced by cefotaxime: a report of two cases

We report two cases, one of a 52-year-old man and one of a 32-year-old man, who were treated with cefotaxime. On day 23 and day 28 of the treatment, respectively, the patients manifested clinically with fever, pruriginous skin rash, and facial edema. Blood tests showed marked eosinophilia and atypical lymphocytosis for both patients, and hepatic cytolysis only in the second patient. Cefotaxime was discontinued in both patients; the clinico-biological picture improved gradually and completely disappeared approximately 4 weeks later. Six weeks after complete recovery, both patients underwent intradermal testing which was positive to cefotaxime (2 mg/ml) at the 48-hour reading and negative to benzylpenicillin, amoxicillin, and cefazolin at the 20-minute and 48-hour readings. These clinical pictures suggest drug rash with eosinophilia and systemic symptoms (DRESS) induced by cefotaxime. To the best of our knowledge, only one case of cefotaxime-induced DRESS has been reported in the medical literature. Thus, we add two new cases of cefotaxime-induced DRESS and emphasize the usefulness and safety of intradermal testing in establishing the diagnosis.     

应用FISH技术检测嗜酸性粒细胞增多症PDGFRA基因异常表达

本研究通过荧光原位杂交（HSH）检测,观察PDGFRA基因在嗜酸性粒细胞增多症中的异常表达情况.应用HSH技术和4q12三色重排探针,检测13例嗜酸性粒细胞增多患者PDGFRA基因的重排形式,同时选择15例正常对照用于确定4q12探针异常荧光信号的正常阈值.结果表明：13例患者中有1例纠正诊断为慢性粒细胞白血病,其余12例中有2例检出FIP1L1-PDGFRA （F/P）融合基因,检出率为17％,但本研究未发现累及PDGFRA基因的其他易位形式.结论：利用4q12三色探针的HSH检测可同时观察PDGFRA基因的多种易位情况,为嗜酸性粒细胞增多症的诊断以及治疗方案的选择提供重要信息.     

Erosive cheilitis as an early manifestation in DRESS syndrome

Drug reaction with eosinophilia and systemic symptoms (DRESS) is a distinct part of severe cutaneous adverse reactions (SCARs). It is characterized by fever, rash, hematologic abnormalities, lymphadenopathy, or/and different degrees of visceral organ involvement. Its diagnosis is particularly challenging due to the variability of its clinical presentations and its long latency period (2–6 weeks). Allopurinol, an uric acid‐lowering drug, has been incriminated in several cases of allopurinol‐induced DRESS syndrome. Through this paper, we present a case of allopurinol‐induced DRESS syndrome with initial oral mucosal involvement. A 69‐year‐old female patient presented with an erosive cheilitis that started 1 week prior to his presentation. The cheilitis was associated with maculopapular rash and fever. She started taking allopurinol, as treatment of Gout, 6 weeks before hospitalization. The histologic findings obtained from skin biopsy were consistent with a toxic drug reaction. A complete blood count (CBC) showed a moderate eosinophilia. Alteration of renal function was also noted, and the diagnosis of allopurinol‐induced DRESS syndrome was made. Systemic corticosteroid therapy was therefore started. The patient completely recovered and had been healthy for 3 years before developing a recurrence after re‐challenge with allopurinol.