Deposits of eosinophil granule proteins in eosinophilic cholecystitis and eosinophilic colitis associated with hypereosinophilic syndrome

Summary A case of hypereosinophilic syndrome with eosinophilic colitis, eosinophilic cholecystitis, and increased serum levels of interleukin-5 (IL-5) and soluble interleukin-2 receptor (sIL-2R) is reported. Immunohistochemical studies of cholecystectomy and colon biopsy specimens with monoclonal antibodies, which are specific for activated eosinophils, secreted eosinophil cationic protein (ECP) and for major basic protein (MBP), demonstrated the presence of numerous activated eosinophils, secretion of ECP, and deposition of MBP in areas of tissue damage. These findings suggest that in eosinophilic cholecystitis and eosinophilic colitis, activated eosinophils infiltrate and degranulate in each tissue, releasing eosinophil granule proteins that produce tissue damage.     

The hypereosinophilic syndrome presenting with eosinophilic mastitis

A case of the hypereosinophilic syndrome presenting with eosinophilic mastitis is described. The histopathology of the mastitis is illustrated and the patient's clinical course reported.     

Successful treatment of ulcerative colitis associated with hypereosinophilic syndrome/chronic eosinophilic leukemia

A 23-year-old female was diagnosed as having simultaneous ulcerative colitis (UC) relapse and hypereosinophilic syndrome (HES)/chronic eosinophilic leukemia (CEL) without FIP1L1-platelet-derived growth factor receptor alpha (PDGFRA) (F/P) fusion gene. Pathological findings of colon specimens were compatible with UC, however, focal severe infiltration of eosinophils was observed in the rectum, which is unusual in UC, suggesting eosinophil-mediated organ damage. Although imatinib mesylate (IM) is usually ineffective for the treatment of HES/CEL with negative-F/P fusion gene, in the present case it led to the remission of HES/CEL and UC at a higher drug dosage level (400 mg/day). That suggested the presence of unknown tyrosine kinase abnormalities not yet categorized.     

Idiopathic hypereosinophilic syndrome associated with rheumatoid arthritis. A case report

The idiopathic hypereosinophilic sindrome (HES) is a disease characterized by persistent blood eosinophilia (> 1500 eosinophils/mm3 > 6 months) -in absence of other ethiologies for eosinophilia (parasitic, allergic, immunological or malignant diseases)- associated with multiple organ involvement (heart, lung, central nervous system, skin, bone marrow, gastrointestinal tract). Reports on rheumatologic manifestations in patients with HES are very rare. In the case we report a typical rheumatoid arthritis developed in a 58-year-old woman with HES treated with glucocorticoids. Because of the marked glucocorticoids side effects shown by the patient (cushingoid habitus, hyperglycemia), we stopped this treatment and replaced it at first by methotrexate and later by cyclosporin, both of them associated with sulfasalazine. These drugs revealed very efficacious both on articular pathology and on the clinical and laboratory manifestations of HES. These data suggest that common pathogenetic mechanisms are likely acting in rheumatoid arthritis and idiopathic hypereosinophilic syndrome.     

Budd-Chiari syndrome associated with hypereosinophilic syndrome; a case report

A 27-year-old man was admitted due to abdominal fullness. He had ascites and subcutaneous nodules on his head, with liver dysfunction and eosinophilia. Abdominal imaging revealed obstruction of the hepatic veins and stenosis of the inferior vena cava. Histological diagnosis of a subcutaneous nodule revealed obstructive thrombophlebitis with eosinophils. Tyrosine kinase created by fusion of the FIP1L1 and PDGFRA genes, which is characteristic of hypereosinophilic syndrome (HES), was detected. He was diagnosed with Budd-Chiari syndrome associated with HES. Liver function tests improved after interventional therapy followed by steroid therapy. It is important to diagnose the cause of Budd-Chiari syndrome.     

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.     

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.     

The idiopathic hypereosinophilic syndrome and chronic eosinophilic leukemia

Idiopathic hypereosinophilic syndrome is a heterogenous group of hematological disorders characterized by eosinophilia (> 1.5 x 10(9)/l) persistent for more than 6 months, exclusion of reactive eosinophilia from other causes, such as parasitic infections or allergy, and evidence of end-organ damage. According to World Health Organization the exclusion includes all neoplastic disorders in which eosinophils are part of the neoplastic clone. Excluded should be also T cell population with aberant phenotype and abnormal cytokine production, recently considert also as "lymphocytic" variants of the HES [42]. HES has to be reclassified as chronic eosinophilic leukemia (CEL) when there is evidence for clonality based on the presence of chromosomal abnormalities or inactivation of X-chromosome in female patients. The successful empiric treatment of patients with tyrosine kinase inhibitor imatinib (Glivec) suggested the presence of an imatinib-sensitive tyrosine kinase inhibitor. The identification of a specific intersticial chromosome deletion del(4)(q12;q12) creating the FIP1L1-PDGFRA fusion gene confirmed this hypothesis. Patients carrying this gene should be reclassified as CEL and detection of this gene is a positive predictor for response to imatinib therapy. Effective doses of imatinib are 100 mg/day. The side effects are minimal. The only exception is an acute left ventricular dysfunction which has been reported in three patients within the first week of treatment with imatinib. Imatinib has been successfully used also in some patients with the constitutively activated thyrosine kinase ETV6-PDGFRbeta [1] and in systemic mast cell disease associated with eosinophilia. Other therapeutical options for HES/CEL have been mentioned. The resistence to imatinib and the possibilities how to overcome it are discussed.     

Fatal Loeffler's endocarditis due to hypereosinophilic syndrome

Hypereosinophilic syndrome (HES) is a rare disorder that can manifest in various organ systems. We report the case of a 54-year-old woman with a remote history of seizure disorder who presented with early signs of right-sided heart failure. Laboratory studies showed significant eosinophilia (8 x 10(9) l(-1)). Computed tomography showed heterogeneity of the liver, mild ascites, moderate pleural effusion, multiple small pulmonary emboli, and a large right ventricular mass. Cardiac magnetic resonance imaging demonstrated that the right ventricular mass was due to thrombus and extensive endomyocardial fibrosis, consistent with Loeffler's endocarditis. Bone marrow biopsy showed marked eosinophilia but no abnormal myeloid maturation or a lymphoproliferative disorder; flow cytometry showed no clonality. Extensive infectious, immunologic, and toxicological studies were negative. Despite resolution of peripheral eosinophilia with medical management, including corticosteroids and cytotoxic agents, anticoagulation for pulmonary emboli and ventricular thrombus, and conventional treatment for heart failure, she developed worsening anasarca and died from ventricular fibrillation within 4 weeks of presentation. Autopsy confirmed the diagnosis. Loeffler's endocarditis, usually a late manifestation of HES, is characterized by fibrous thickening of the endocardium, leading to apical obliteration and restrictive cardiomyopathy, resulting in heart failure, thromboembolic events, or atrial fibrillation. HES is a potentially fatal disease with less than 50% reported 10-year survival. This case presentation is unusual in its rapidly progressive course leading to sudden death.     

Partial Budd-Chiari syndrome associated with a hypereosinophilic syndrome

We report a rare case of hypereosinophilic syndrome associated with a partial obstruction of the hepatic veins (Budd-Chiari syndrome). The case was assessed by ultrasonic examination, computed tomography and magnetic resonance tomography. We postulate an association because endothelium damage and thrombosis is reported in hypereosinophilic syndromes. Elevated liver enzymes in a patient with hypereosinophilic syndrome could be associated with a Budd-Chiari syndrome.     

Idiopathic hypereosinophilic syndrome and "eosinophilic leukemia".

We report a case of idiopathic hypereosinophilia syndrome (H.E.S.) with a pronounced myeloproliferative disorder, which during the course of the illness has exceeded more than one "blastic crisis". This again proposes the difficult relationship between H.E.S. and myeloproliferative syndromes (M.S.), and is indicative of why some cases of H.E.S. are differentially diagnosed as chronic myeloid leukemia (C.M.L.) with an eosinophilic component.     

A case of hypereosinophilic syndrome associated with pulmonary infarction and hepatic vein obstruction (Budd-Chiari syndrome)

A 21-year-old man was admitted in March 1987 with low grade fever and chest pain. Eosinophilia had been pointed out and PIE syndrome was diagnosed in another hospital a month before admission. Steroid therapy had been started. On the first admission, the chest roentgenogram showed bilateral pleural effusion and a nodular shadow in the left lower lung field. Open lung biopsy was performed and a diagnosis of pulmonary infarction was made. Eosinophilia, low grade fever and chest pain were improved by steroid therapy. He was discharged in April 1987. He was readmitted in September 1987 because of fever, back pain and abdominal distension. On the second admission, eosinophilia (4,510/mm3) was pointed out. The case was diagnosed as hepatic vein obstruction by hepatic vein angiography, liver biopsy and ultrasonic examination. He had transient remission on corticosteroid and anti-coagulant therapy. This case was considered as a rare case of hypereosinophilic syndrome associated with pulmonary infarction and Budd-Chiari syndrome.