A case of hypereosinophilic syndrome presenting with intractable gastric ulcers

We report a rare case of hypereosinophilic syndrome (HES) presenting with intractable gastric ulcers. A 71-year-old man was admitted with epigastric pain. Initial endoscopic findings revealed multiple, active gastric ulcers in the gastric antrum. He underwent Helicobacter pylori (H pylori ) eradication therapy followed by proton pump inhibitor (PPI) therapy. However, followup endoscopy at 4, 6, 10 and 14 mo revealed persistent multiple gastric ulcers without significant improvement. The proportion of his eosinophil count increased to 43% (total count: 7903/mm3). Abdominal-pelvic and chest computed tomography scans showed multiple small nodules in the liver and both lungs. The endoscopic biopsy specimen taken from the gastric antrum revealed prominent eosinophilic infiltration, and the liver biopsy specimen also showed eosinophilic infiltration in the portal tract and sinusoid. A bone marrow biopsy disclosed eosinophilic hyperplasia as well as increased cellularity of 70%. The patient was finally diagnosed with HES involving the stomach, liver, lung, and bone marrow. When gastric ulcers do not improve despite H pylori eradication and prolonged PPI therapy, infiltrative gastric disorders such as HES should be considered.     

A case of hypereosinophilic syndrome presenting mid-ventricular obstruction

A 59-year-old man with hypereosinophilic syndrome (HES) who had been maintained with low-dose prednisolone for 5 years developed the characteristic features of hypertrophic cardiomyopathy. Left ventricular endomyocardial biopsy revealed no eosinophilic infiltration but extensive myocardial fibrosis. Cardiac involvement in HES presents as endocardial fibrosis, resulting in a clinical presentation of restrictive cardiomyopathy. HES heart disease can also present dilated cardiomyopathy, but myocardial hypertrophy has only rarely been noted in conjunction with HES. This report concerns a patient with HES who had clinical and hemodynamic evidence of asymmetric septal hypertrophy with mid-ventricular obstruction. Received: August 1, 2001 / Accepted: October 26, 2001     

布加综合征并嗜酸性粒细胞增多综合征1例

一、病例资料 男性患者,34岁,兽医.2002年7月无明显诱因出现腹胀、纳差,于我院介入科诊断为布加综合征,并行介入治疗.介入术后腹胀有所减轻,但仍反复出现,3d前腹胀加重,腹泻3～4次/d,无腹痛,黄色糊状便,无脓血,于2010年12月2日第二次入院.患者无长期饮酒史,无长期或反复用药史,无寄生虫病史,无慢性心脏病史.查体:皮肤、巩膜无黄染,无肝掌、蜘蛛痣,无瘀点、瘀斑及出血点.全身浅表淋巴结未触及肿大.腹膨隆,全腹无压痛、反跳痛,肝脾肋下未及,肝剑下可及边,质韧,无触痛.移动性浊音阳性.     

Cyclosporin for hypereosinophilic syndrome

Summary The idiopathic hypereosinophilic syndrome (HES) comprises a heterogeneous group of disorders with unknown pathogenesis characterized by persistent peripheral blood and bone marrow eosinophilia and eosinophil infiltrates of multiple organs, leading to severe organ dysfunction. Lymphokine-mediated T-lymphocyte control of human eosinophilic granulopoiesis is thought to play a major role in the pathogenesis of HES. Treatment of this disease with cyclosporin-A (CSA) therefore appears to be sensible. We report the case of a patient with a severe HES who failed to respond adequately to glucocorticoid treatment. With additional CSA therapy, disease activity showed a favorable remission and eosinophil counts rapidly decreased to the normal range and remained normal even after reduction of the methylprednisolone dosage to 7.5 mg daily. To date his remission has continued for more than 6 months. In the meantime we have confirmed the beneficial effects of CSA in two other cases of HES. This suggests that CSA treatment is justified, at least in combination with glucocorticoids, in severe cases of HES to prevent the side effects of a longlasting high-dose glucocorticoid therapy.     

嗜酸细胞性胃肠炎与高嗜酸性粒细胞综合征

目的　探讨嗜酸细胞性胃肠炎 (EG)和高嗜酸性粒细胞综合征 (HES)临床特征的异同。方法　回顾性对照研究 9例EG和 19例HES ,分析其临床表现及治疗经过。结果　EG组腹泻、腹痛、体重下降和消化道受累的发生率高于HES组 (P <0 .0 5 )。HES组循环嗜酸性粒细胞 (EOS)和白细胞计数、IgE水平均高于EG组 (P值均 <0 .0 5 )。 9例EG和 3例HES行内镜黏膜活检发现有EOS浸润 ,HES的大肠黏膜活检阳性率明显高于EG(P <0 .0 1)。EG患者经对症或激素治疗均缓解。激素和化疗药物治疗HES有效 (12 / 15 )。结论　EG是以胃肠道组织中EOS异常浸润为特征的胃肠道疾病。循环中EOS、白细胞计数和IgE的高水平以及大肠的EOS浸润提示HES的诊断。     

Idiopathic hypereosinophilic syndrome presenting with hepatitis and achalasia

Idiopathic hypereosinophilic syndrome (HES) is a rare diagnosis defined by the World Health Organization as a persistent eosinophilia for 6 months and resulting in end-organ dysfunction. While many patients present with nonspecific symptoms, others will present with symptoms of the affected organs, most commonly those involving the heart, skin, or nervous system. Gastrointestinal or liver involvement is estimated to affect up to one-third of patients with HES, although patients with clinically significant disease are limited to case reports. This is the first report of a patient presenting with hepatitis and achalasia related to idiopathic HES.     

Spontaneous liver rupture in hypereosinophilic syndrome： A rare but fatal complication

We report a rare case of spontaneous liver rupture in a patient with hypereosinophilic syndrome (HES), of which the diagnosis was delayed, resulting in a fatal outcome. The diagnostic criteria and treatment of HES with hepatic involvement were reviewed. The possible cause of spontaneous liver rupture in HES and its management were also discussed. To our knowledge, this is the first case report of spontaneous liver rupture in HES. We emphasized the need of a high index of suspicion in diagnosing HES, so that early treatment could be initiated.     

Granular acute lymphoblastic leukemia with hypereosinophilic syndrome

9 /l), features of hypereosinophilic syndrome, and acute lymphoblastic leukemia (ALL-L2), the latter characterized by the presence of granular blasts. Blasts were negative for myeloperoxidase, non-specific esterase, acid phosphatase, periodic-acid Schiff stain, and toluidine blue. They exhibited an early pre-B immunophenotype (TdT, CD19, CD10, CD20 and CD22 positive) and stained negative for T (CD7, CD2, CD5 and CD3) and myeloid markers (MPO, CD33 and CD13). Chromosomal analysis revealed a normal karyotype. To the best of our knowledge, this case represents the first report of the coexistence of granular ALL and hypereosinophilic syndrome. Received: 11 May 1999 / Accepted: 20 October 1999     

Eosinophilic myocarditis and hypereosinophilic syndrome

Hypereosinophilic syndrome (HES) is a heterogeneous group of hematological disorders characterized by a chronic, unexplained hypereosinophilia with tissue damage. Cardiac involvement occurs in ～20% of patients with HES and represents a major turning point. Cardiac injuries related to eosinophilia are divided into three chronological phases: eosinophilic infiltration, thrombosis, and fibrosis. We report a case of a 33-year-old woman diagnosed with HES, with pulmonary and gastrointestinal involvement and eosinophilic myocarditis in cardiogenic shock. The evolution was favorable with dobutamine, anticoagulation, corticosteroids, and later, β-blockers and angiotensin-converting enzyme inhibitors. Cardiac involvement in HES is rare but carries a poor prognosis. Corticosteroids are considered by many to be the mainstay of treatment. Although new treatments have been suggested, only a few seem promising.     

Reversal of cardiac abnormalities in a young man with idiopathic hypereosinophilic syndrome using a tyrosine kinase inhibitor.

We report a case of idiopathic hypereosinophilic syndrome in a young man, who showed symptoms and electrocardiographic findings mimicking an acute coronary syndrome. Two-dimensional echocardiography, together with laboratory data, allowed us to make the diagnosis and to start a treatment with imatinib mesylate, a 2-phenylamonopyrimidine-based tyrosine kinase inhibitor, which reversed the cardiac abnormalities.     

A case of hypereosinophilic syndrome with asymmetric septal hypertrophy

A case of idiopathic hypereosinophilic syndrome (HES) with asymmetric septal hypertrophy (ASH) is described: this is a very rare association. The patient was a 56-year-old male with hypereosinophilia lasting for 10 years. The white blood cell count was 11200/mm³, with 22% eosinophils, and eosinophilic hyperplasia (7.2%) was noted in the bone marrow. A peripheral blood smear showed vacuolated eosinophils with a reduced content of granules. An ultrastructure study of the eosinophils revealed reduced numbers of crystalloid granules which appeared to be dissolving with reversal of normal staining. An echocardiogram and a biventriculoglam indicated ASH with the interventricular septal wall thickness of 2.4 cm and the left ventricular posterior wall thickness of 1.5 cm. Right ventricular endomyocardial biopsy revealed no eosinophilic infiltration, but endocardial thickening, subendocardial fibrosis, hypertrophy, myocytolysis, and fragmentation of muscle bundles were observed.     

Successful Treatment of Idiopathic Hypereosinophilic Syndrome with Imatinib Mesylate: A Case Report

Patients with idiopathic hypereosinophilic syndrome (HES) show persistent hypereosinophilia of unknown etiology that is associated with end-organ damage. Different treatments, including the use of corticosteroids and cytotoxics, have been investigated for HES with modest success. We describe a patient with HES who had significant end-organ damage from hypereosinophilia and remained refractory to conventional therapy. Therapy with imatinib mesylate, a selective tyrosine kinase inhibitor that is highly effective in treating patients with BCR-ABL-positive chronic myeloid leukemia, was tried with the patient. The result was impressive, with hematologic remission achieved after 12 days of administration. Our finding concurs with recent reports that imatinib mesylate may be a promising agent in the treatment of some cases of HES.     

Idiopathic hypereosinophilic syndrome associated with multiple intracardiac thrombi

A 17-year-old man was referred for dyspnea, fatigue, and fever. Idiopathic hypereosinophilic syndrome was diagnosed. Transthoracic echocardiography demonstrated multiple intracardiac thrombi in the left ventricular apex. Dissolution of thrombi was not seen despite intensive medical therapy. The patient died because of cerebral embolus.     

高嗜酸性粒细胞综合征2例报告并文献复习

目的:探讨高嗜酸性粒细胞综合征的诊断及治疗。方法:选取高嗜酸性粒细胞综合征患者2例,对其FIP1L1-PDGFRA融合基因进行检测,结合临床资料进行分析,并复习相关文献。结果:2例中第1例FIP1L1-PDGFRA融合基因为阳性,确诊为慢性嗜酸性粒细胞白血病(CEL),应用甲磺酸伊马替尼治疗效果好,另1例FIP1L1-PDGFRA融合基因阴性,诊断为特发性嗜酸性粒细胞综合征,用糖皮质激素治疗有效。结论:对FIP1L1-PDGFRA融合基因的检测,并结合临床,有助于高嗜酸性粒细胞综合征的诊断及鉴别诊断,并对指导治疗有一定的意义。     

Idiopathic hypereosinophilic syndrome in hemodialysis patients: Case reports

Rationale:Herein, we report 3 hemodialysis patients with idiopathic hypereosinophilic syndrome who were successfully treated using corticosteroid therapy.Patient concerns:Case 1 was a 63-year-old man who was undergoing hemodialysis because of bilateral nephrectomy and developed hypereosinophilia with digestive symptoms, myocardial injury, and intradialytic hypotension. Case 2 was an 83-year-old man who was undergoing hemodialysis because of nephrosclerosis and developed hypereosinophilia with pruritus, myocardial injury, and intradialytic hypotension. Case 3 was a 59-year-old man who was undergoing hemodialysis because of diabetic nephropathy and developed hypereosinophilia with pruritus, myocardial injury, and intradialytic hypotension.Diagnoses:All 3 patients presented with hypereosinophilia (eosinophil count ≥1500 /μL for more than 1 month) and multiple-organ involvement (intradialytic hypotension, cardiac injury, digestive symptoms, and allergic dermatitis). A specific cause for the hypereosinophilia was not identified by systemic computed tomography, electrocardiography, echocardiography, bone marrow examination, or blood tests. Furthermore, Case 2 and 3 had not recently started taking any new drugs and drug-induced lymphocyte stimulation tests were negative in Case 1. Therefore, they were diagnosed with idiopathic hypereosinophilic syndrome.Interventions:All 3 patients received corticosteroid therapy with prednisolone at a dose of 40 mg/d, 30 mg/d, and 60 mg/d in Case 1, 2, and 3, respectively.Outcomes:Their digestive symptoms, pruritus, intradialytic hypotension, and serum troponin I concentrations were immediately improved alongside reductions in their eosinophil counts.Lessons:There have been few case reports of idiopathic hypereosinophilic syndrome in patients undergoing hemodialysis. We believe that recording of the clinical findings and treatments of such patients is mandatory to establish the optimal management of idiopathic hypereosinophilic syndrome.     

高嗜酸粒细胞综合征心血管损害的临床分析

目的 分析高嗜酸粒细胞综合征(HFS)心血管损害的临床特点.方法 收集北京协和医院149例HES患者的临床资料,分析心血管损害的发生率及类型.结果 149例HES患者中,心血管受累患者共49例(32.9%),其中女性15例,男性34例,年龄1.5～78.0(41.3±16.9)岁,病程14 d～164个月(26.4±72.3)个月.其心血管受累分别表现为心电图ST-T异常或心律失常、心肌或心内膜损害、心内血栓形成、心包积液、肺动脉高压、瓣膜病变、动脉或静脉血栓形成和其他心血管损害.49例患者中11例(22.4%)经对症治疗后症状有改善但仍反复发作,3例(6.1%)患者治疗无效死亡.与单纯非特异性心电图异常患者(21例)相比,存在超声心动图异常的HES者(28例)中有更多患者需要接受激素和免疫抑制剂的联合治疗(前者使用单一激素与联合治疗分别为15例、6例,后者分别为16例、12例,P=0.044)且预后更差(两组死亡例数分别为0例与3例,P=0.029).结论 心血管损害是HES的常见并发症,表现复杂多样,超声心动图评估心血管受累简单有效,心血管受累是提示预后的危险因素.     

Pitfalls in the diagnosis of hypereosinophilic syndrome: a report of two cases

The idiopathic hypereosinophilic syndrome is empirically defined as the presence of prolonged eosinophilia without 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 two cases where the diagnosis of hypereosinophilic syndrome was proposed although the classic criteria were not met. In the first case total peripheral eosinophil counts were relatively low, but pathological evidence clearly showed infiltration of eosinophils in the damaged tissues. An hypothesis to explain this discrepancy is formulated. The second case did not fulfil the first feature either, although the clinical presentation and disease course corresponded well with other cases reported in the literature. The delay in diagnosis was caused by early institution of corticosteroids, clearing all evidence of eosinophil involvement in the observed tissue damage.     

Imatinib mesylate as a novel treatment option for hypereosinophilic syndrome: two case reports and a comprehensive review of the literature

Hypereosinophilic syndromes (HES) are a heterogenous group of rare disorders characterized by sustained and otherwise unexplained overproduction of eosinophils with organ involvement and consecutive dysfunction. Recent reports document the efficacy of imatinib mesylate in a large proportion of HES patients (65%). Rearrangements involving the platelet-derived growth factor receptor genes (PDGFRA and PDGFRB), both tyrosine kinase receptors, have been demonstrated to be pathogenetically linked to the dysregulated clonal overproduction of eosinophils. This refined hypothesis has been confirmed by the discovery of the novel FIP1L1–PDGFRA fusion gene, which is a gain-of-function gene on chromosome 4q12. Its product is an imatinib-sensitive tyrosine kinase, which can be found in a subset of patients with HES, particularly in those responding to treatment with imatinib mesylate. Here, we sum up recent knowledge of clinical features, pathophysiology and novel treatment aspects of HES by performing a comprehensive search of the available literature and report on 94 patients. We particularly address the issue of organ involvement and specific characteristics of the variable clinical pictures. In addition, two cases will be presented, which illustrate typical clinical scenarios and treatment outcome. A.M.S. Müller and U.M. Martens contributed equally to the paper.     

肝移植术后腹腔高压致腹腔间隔室综合征2例

腹腔高压可导致多器官功能障碍甚至衰竭,演进成致死性腹腔间隔室综合征.肝移植存在导致腹腔高压的多种病理因素,一旦形成腹腔间隔室综合征,预后极差.本文报道肝移植术后腹腔间隔室综合征2例,结合文献资料对该病症进行分析讨论,以提高对该病症的认识,改善预后.     

以胸腔积液为首发症状的特发性嗜酸粒细胞增多综合征1例并文献复习

目的 探讨以胸腔积液为首发症状的特发性嗜酸粒细胞增多综合征(idiopathichypereosinophilic syndrome,IHES)的临床特点及诊治经验,提高广大临床工作者对该病的认识.方法 回顾性分析重庆医科大学附属第一医院呼吸内科收治的1例IHES患者的病例资料,并结合国内外文献进行文献复习.以“特发性嗜酸粒细胞增多综合征”为检索词检索中国期刊全文数据库(CNKI)、中文科技期刊数据库(维普)、万方数据库,以“idiopathichypereosinophilic syndrome”、“eosinophilia”、“hypereosinophilia”为检索词检索Embase数据库、MEDLINE数据库(PubMed),检索时间为1990年1月到2015年12月.结果 患者为青年男性,2015年10月10日因“反复咳嗽、胸闷、气促2个月,加重半个月”入院,血常规提示嗜酸粒细胞绝对值17.28×109/L,胸部CT提示左侧胸腔中量积液,右侧胸腔少量积液,最终被诊断为IHES,糖皮质激素治疗2个月后,患者临床症状好转,胸腔积液明显吸收.文献经检索、去重后,共筛选诊断为IHES的国内外病例报道74篇,其中合并胸腔积液的病例报道共25篇.国内已公开报道IHES患者共103例,最常受累的部位包括皮肤(约34.0％),其次为呼吸系统、消化系统,14.6％伴有胸腔积液.结论 IHES的临床症状常不典型,易与其他疾病相混淆,诊断较为复杂,只有提高临床工作者对于该病的认识及其临床资料的掌握,才能真正减少误诊误治.