Eosinophilic cystitis mimicking bladder cancer-considerations on the management based upon a case report and a review of the literature.

The hypereosinophilic syndrome (HES) is a rare disorder characterized by hypereosinophilia and infiltration of various organs with eosinophils. Eosinophilic cystitis (EC), mimicking bladder cancer clinically but also in ultrasound and in radiographic imaging, is one potential manifestation of the HES occurring in adults as well as in children. This case report describes the course of disease in a 57-year-old male presenting with severe gait disorders and symptoms of a low compliance bladder caused by a large retropubic tumor. After extensive urine and serologic examination and histologic confirmation of EC the patient was subjected to medical treatment with cetirizine and prednisolone for 5 weeks. While gait disorders rapidly resolved, micturition normalized only 10 months after initiation of therapy. Based upon this course the authors recommend patience and reluctance concerning radical surgical intervention in EC. Key Points • Eosinophilic cystitis is a rare condition with app. 200 cases reported, so far. • Etiology of eosinophilic cystitis is obscure, but allergies and parasitic infections may trigger the disease. • Genetic alterations (e.g., BRAF mutations) may predispose for the disease • Corticosteroids and antihistamines are the backbone of therapy and may be complemented by antibiotics and non-steroidal anti-inflammatory drugs in case of concomitant (underlying) infections. • As recovery can occur even after a long time, radical surgery should be restricted to highly selected cases.

Missed hypereosinophilic syndrome in a critically ill patient with systemic lupus erythematosus.

A high functioning 74-year-old man with systemic lupus erythematosus presented to the emergency department with acute anxiety. He was found to have elevated cardiac enzymes and admitted to the cardiology service for investigation. In hospital, he developed an erythematous papular rash, and deteriorated to being somnolent and bedridden. He was found to have new multiterritory ischaemic strokes. It was eventually noted that he had persistent eosinophilia, present even on admission, which had been overlooked as the total leucocyte count was normal. Serology for antiphospholipid antibody syndrome (APS) was positive. He was diagnosed with hypereosinophilic syndrome (HES) secondary to new APS, and responded to high-dose steroids. This case highlights the importance of fully evaluating a leucocyte differential to make a diagnosis of HES. We discuss the definition, clinical manifestations, diagnostic approach and management of this important condition.

Central Nervous System and Cardiac Involvement in the Hypereosinophilic Syndrome: A Case Report.

Hypereosinophilic syndrome is a rare entity and heterogeneous group of disorders characterized by hypereosinophilia and organ involvement. In this study, we presented a 49-year-old woman with cardiac tamponade in the context of Hypereosinophilic syndrome. Identifying hypereosinophilia as the underlying cause can have tremendous clinical implications for rapid initiation of appropriate treatment to minimize further end organ damage.

Left ventricular rotational mechanics in hypereosinophilic syndrome-Analysis from the three-dimensional speckle-tracking echocardiographic MAGYAR-Path Study.

INTRODUCTION: Hypereosinophilic syndrome (HES) is a very heterogeneous group of disorders with varied etiologies characterized by peripheral eosinophilia and eosinophilic tissue/end-organ damage. Three-dimensional speckle-tracking echocardiography (3DSTE) was used for assessment of left ventricular (LV) rotational mechanics in HES patients. METHODS: The study comprised 13 HES patients, from which one patient was excluded due to insufficient image quality. The remaining patient population consisted of 12 HES cases (mean age: 59.7 ± 13.7 years, eight males). The control group consisted of 36 healthy volunteers (mean age: 52.9 ± 8.3 years, 23 males). 3DSTE was used for the evaluation of LV rotational abnormalities. RESULTS: Both LV apical rotation (4.86 ± 1.92 degree vs 10.07 ± 3.92 degree, P < .0001) and LV twist (8.52 ± 2.79 degree vs 14.41 ± 4.26 degree, P < .0001) showed significant deteriorations in most of HES patients. Time-to-peak LV apical rotation (380 ± 115 ms vs 344 ± 69 ms, P = .56), LV basal rotation (335 ± 148 ms vs 337 ± 111 ms, P = .89), and LV twist (348 ± 91 ms vs 320 ± 60 ms, P = .64) were not significantly different between HES patients and controls. No correlations could be detected between absolute eosinophil count and eosinophil ratio and apical LV rotation (r = 0.12, P = .51 and r = 0.23, P = .45, respectively) and LV twist (r = 0.24, P = .39 and r = 0.31, P = .34, respectively). In two subjects, the absence of LV twist called LV "rigid body rotation" (RBR) was detected. CONCLUSIONS: Reduced LV apical rotation and twist could be demonstrated in HES. LV-RBR could be detected in some HES patients.

Hypereosinophilic syndrome in an infant / Síndrome hipereosinofílico en un lactante

Abstract Background The hypereosinophilic syndrome (HES) is defined by an eosinophilic count > 1500 cell/mm3 and organ damage or dysfunction that can be easily mistaken for atopic dermatitis or pulmonary pathologies. Timely diagnosis and treatment can improve the prognosis and avoid heart and renal complications or lung fibrosis. Case report The case of an infant is reported with a 24-h evolution of cough and fever, personal history of atopic dermatitis, and a generalized dermatosis 2 months earlier. In the initial approach, respiratory disease was considered. However, blood count reported hypereosinophilia, which led to further studies and the diagnosis of the HES. Conclusions Although a rare pathology, it is important to consider the HES in children with common symptoms, and unusual evolution or poor treatment response and persistent hypereosinophilia.

Resumen Introducción El síndrome hipereosinofílico se define por la cuenta de eosinófilos > 1,500 células/mm3 con daño orgánico o disfunción, sin ninguna causa subyacente. Puede ser fácilmente confundido con una dermatitis atópica o con patologías pulmonares. El diagnóstico temprano y el tratamiento adecuado pueden mejorar el pronóstico y evitar complicaciones cardíacas y renales o el desarrollo de fibrosis pulmonar. Caso clínico Se reporta el caso de un lactante con tos y fiebre de 24 horas de evolución y una historia personal de dermatitis atópica, además de dermatosis generalizada dos meses antes. Inicialmente, se consideró como una enfermedad respiratoria; sin embargo, la cuenta de células sanguíneas reportó hipereosinofilia, lo cual condujo a estudios confirmatorios y al diagnóstico de síndrome hipereosinofílico. Conclusiones A pesar de ser una enfermedad rara, es de suma importancia considerar el síndrome hipereosinofílico en el diagnóstico diferencial en niños con una evolución atípica o con pobre respuesta al tratamiento, además de hipereosinofilia persistente.

Hypereosinophilic syndrome in an infant.

Background: The hypereosinophilic syndrome (HES) is defined by an eosinophilic count > 1500 cell/mm3 and organ damage or dysfunction that can be easily mistaken for atopic dermatitis or pulmonary pathologies. Timely diagnosis and treatment can improve the prognosis and avoid heart and renal complications or lung fibrosis. Case report: The case of an infant is reported with a 24-h evolution of cough and fever, personal history of atopic dermatitis, and a generalized dermatosis 2 months earlier. In the initial approach, respiratory disease was considered. However, blood count reported hypereosinophilia, which led to further studies and the diagnosis of the HES. Conclusions: Although a rare pathology, it is important to consider the HES in children with common symptoms, and unusual evolution or poor treatment response and persistent hypereosinophilia.

Introducción: El síndrome hipereosinofílico se define por la cuenta de eosinófilos > 1,500 células/mm3 con daño orgánico o disfunción, sin ninguna causa subyacente. Puede ser fácilmente confundido con una dermatitis atópica o con patologías pulmonares. El diagnóstico temprano y el tratamiento adecuado pueden mejorar el pronóstico y evitar complicaciones cardíacas y renales o el desarrollo de fibrosis pulmonar. Caso clínico: Se reporta el caso de un lactante con tos y fiebre de 24 horas de evolución y una historia personal de dermatitis atópica, además de dermatosis generalizada dos meses antes. Inicialmente, se consideró como una enfermedad respiratoria; sin embargo, la cuenta de células sanguíneas reportó hipereosinofilia, lo cual condujo a estudios confirmatorios y al diagnóstico de síndrome hipereosinofílico. Conclusiones: A pesar de ser una enfermedad rara, es de suma importancia considerar el síndrome hipereosinofílico en el diagnóstico diferencial en niños con una evolución atípica o con pobre respuesta al tratamiento, además de hipereosinofilia persistente.

Gastrointestinal Manifestations of Hypereosinophilic Syndromes and Mast Cell Disorders: a Comprehensive Review.

Hypereosinophilic syndrome and mastocytosis are relatively rare proliferative diseases encountered in the general population. However, allergists frequently consider these disorders in the differential of patients presenting with gastrointestinal, pulmonary, cutaneous, and allergic symptoms. Gastrointestinal symptoms are some of the most frequent and/or debilitating aspects of both disease states and in many cases lead to poor quality of life and functional limitation for the patient. They are the third most common clinical manifestation in hypereosinophilic syndrome and have been found to be the most distressful aspect of the disorder in those with systemic mastocytosis. Both eosinophils and mast cells play integral parts in normal gut physiology, but when and how exactly their effector functionality translates into clinically significant disease remains unclear, and the available literature regarding their pathophysiology remains sparse. Eosinophils and mast cells even, in fact, may not necessarily function in isolation from each other but can participate in bidirectional crosstalk. Both are affected by similar mediators and can also influence one another in a paracrine fashion. Their interactions include both production of soluble mediators for specific eosinophil and mast cell receptors (for example, eosinophil recruitment and activation by mast cells releasing histamine and eotaxin) as well as direct physical contact. The mechanistic relationship between clonal forms of hypereosinophilia and systemic mastocytosis has also been explored. The nature of gastrointestinal symptomatology in the setting of both hypereosinophilic syndrome and mast cell disease is frequently manifold, heterogeneous, and the lack of better targeted therapy makes diagnosis and management challenging, especially when faced with a substantial differential. Currently, the management of these gastrointestinal symptoms relies on the treatment of the overall disease process. In hypereosinophilia patients, systemic corticosteroids are mainstay, although steroid-sparing agents such as hydroxyurea, IFN-α, methotrexate, cyclosporine, imatinib, and mepolizumab have been utilized with varying success. In mastocytosis patients, anti-mediator therapy with antihistamines and mast cell stabilization with cromolyn sodium can be considered treatments of choice, followed by other therapies yet to be thoroughly studied, including the role of the low-histamine diet, corticosteroids, and treatment of associated IBS symptoms. Given that both eosinophils and mast cells may have joint pathophysiologic roles, they have the potential to be a combined target for therapeutic intervention in disease states exhibiting eosinophil or mast cell involvement.

Thrombosis in the portal venous system caused by hypereosinophilic syndrome: A case report.

RATIONALE: Extensive thrombosis in the portal venous system caused by hypereosinophilic syndrome (HES) is rare, and there is no consensus on anticoagulant and thrombolytic treatments for arteriovenous thrombosis caused by HES. PATIENT CONCERNS: The clinical data of a patient with extensive thrombosis in his portal venous system (superior mesenteric, splenic, hepatic, and portal veins), renal artery thrombosis, and mesenteric thrombosis caused by HES with secondary gastrointestinal bleeding and intestinal necrosis were retrospectively analyzed. Before admission, his eosinophil count increased to 7.47 × 10/L, and HES had been confirmed via bone marrow cytology. The patient experienced fever, cough, abdominal pain, massive hematemesis, and hematochezia that developed in succession. Abdominal computed tomography showed portal vein and superior mesenteric vein thromboses. DIAGNOSIS: Hypereosinophilic syndrome; extensive thrombosis in the portal venous system; acute eosinophil-associated pneumonia; gastrointestinal bleeding; intestinal necrosis. INTERVENTIONS: The patient was first treated with methylprednisolone, plasma exchange/hemofiltration, and single or combined use of unfractionated heparin and argatroban for anticoagulation. He was also administered alteplase and urokinase, successively, for thrombolytic treatment. Once the thromboses finally disappeared, the patient underwent surgery to excise a necrotic intestinal canal. OUTCOMES: The thromboses disappeared with these treatments, and the patient recovered after the necrotic intestinal canal was excised. LESSONS: The clinical manifestations of HES are complex and varied, and this condition can cause severe and extensive arteriovenous thrombosis. Anticoagulation therapy and thrombolysis are necessary interventions, and appear to be safe and effective.

Loeffler endocarditis as a rare cause of heart failure with preserved ejection fraction: A case report and review of literature.

RATIONALE: Hypereosinophilic syndrome (HES) is a rare disease characterized by hypereosinophilia and its ensuing organ damage. Cardiac involvement is divided into 3 chronological stages: an acute necrotic stage; a thrombus formation stage; and a fibrotic stage. Infiltration of the myocardium by eosinophilic cells followed by endomyocardial fibrosis is known as "Loeffler endocarditis." PATIENT CONCERNS: We report a case of a 60-year-old man diagnosed with left-sided restrictive cardiomyopathy. DIAGNOSIS: The patient experienced heart failure with preserved ejection fraction. The cardiac MRI showed intense, linear, delayed gadolinium enhancement of the endocardium of the lateral wall of the left ventricle, and obliteration of the LV apex. He was ultimately identified as Loeffler endocarditis. INTERVENTION: A bone marrow smear and biopsy revealed the FIP1L1-PDGFRA fusion gene was positive in 82% of segmented nucleated cells. OUTCOME: Our patient responded well to prednisone at 1 mg/kg/d. LESSONS: HES is a rare disease that often afflicts the heart. Cardiac involvement in hypereosinophilia, especially Loeffler endocarditis, carries a poor prognosis and significant mortality. Early detection and treatment of the disease is therefore essential. Further studies are needed to ascertain therapeutic corticosteroid dosages and develop targeted gene therapies, both important steps to ameliorate the effects of Loeffler endocarditis and improve patient outcomes.

A Case of Loeffler Endocarditis That Showed Endomyocardial Systolic Dysfunction Detected by Layer Specific Strain Analysis.

Loeffler endocarditis is a rare comprehensive cardiac manifestation caused by eosinophilic cell infiltrations and is present in 50%-60% of patients with hypereosinophilic syndrome (HES). Left ventricle (LV) endocardial systolic dysfunction is a major cause of morbidity and mortality in HES and Loeffler endocarditis. We present a case of Loeffler endocarditis, whose left ventricular (LV) systolic dysfunction and endocardial systolic dysfunction were first neglected by conventional transthoracic echocardiography (TTE), but were later pointed out by layer-specific longitudinal strain analysis. With timely initial therapeutic management, the patient's outcome was remarkable. Thus, we strongly recommend strain analysis as a necessary supplementary test of conventional TTE in all patients with Loeffler endocarditis.

Usefulness of three-dimensional echocardiography in the assessment of valvular involvement in Loeffler endocarditis.

Loeffler endocarditis is a complication of hypereosinophilic syndrome resulting from eosinophilic infiltration of heart tissue. We report a case of Loeffler endocarditis in which three-dimensional transthoracic and transesophageal echocardiography provided additional information to what was found by two-dimensional transthoracic echocardiography alone. Our case illustrates the usefulness of combined two- and three-dimensional echocardiography in the assessment of Loeffler endocarditis. In addition, a summary of the features of hypereosinophilic syndrome and Loeffler endocarditis is provided in tabular form.

Loeffler endocarditis in young woman - a case report.

Loeffler endocarditis is a rare acquired endocardial and myocardial disease characterized by a sharp decrease in the compliance of either or both ventricles with an acute diastolic dysfunction and massive mural thrombosis. This disease is presented in the classification of cardiomyopathies and is a variant of restrictive cardiomyopathy. Today Loeffler endocarditis is considered as a manifestation of hypereosinophilic syndrome with predominant heart involvement. The life-time diagnosis of myocardial injury due to eosinophilic infiltration is rare, or it is diagnosed at the stage of necrotizing endomyocarditis, when the treatment is no longer effective. A number of issues regarding the individual aspects of the pathogenesis of hypereosinophilic syndrome and Loeffler endocarditis are still not fully understood, as well as the long-term prospects for the use of drugs for the treatment of hypereosinophilic syndrome, especially in young and middle-aged persons. Loeffler endocarditis can be suspected in the presence of hypereosinophilia on the background of causeless (unexplainable) hypertrophy of the left ventricle or both ventricles. The article includes a case of the life-time diagnosis of this disease in a young woman with the retrospective analysis of the early stages of the disease, echocardiographic and radiologic imaging at the advanced stage of the disease and quite successful treatment option for this disease.