A case of death in children with hypereosinophilia complicated with Loffler endocarditis / 中国综合临床

Objective:To investigate the clinical features of hypereosinophilia (HE) complicated with Loffler endocarditis in children, in order to analyze the etiology, clinical stage and prognosis of HE complicated with Loffler endocarditis.Methods:A case of childhood hypereosinophilia complicated with Loffler endocarditis diagnosed and treated in the Second Hospital of Hebei Medical University was analyzed retrospectively, and its clinical characteristics, causes and clinical stages were analyzed.Results:In this study, the patient was a young male. He had diarrhea for more than 40 days due to intermittent abdominal pain, dyspnea for 10 days, and poor food intake for 3 days. The blood routine showed that he was HE. The ultrasound showed Loffler endocarditis. The child had severe heart failure and multiple organ functions were involved. After active treatment, he still had multiple organ failure, and finally died of multiple organ failure and ventricular fibrillation.Conclusion:Loffler endocarditis is a serious complication of HE, with low morbidity and rare clinical manifestations. Early intervention can reduce mortality.

Fibrosis Endomiocárdica: Presentación de Caso Clínico y Revisión de la literatura / Endomyocardial Fibrosis: Case Report and Literature Review

La fibrosis endomiocárdica (FEM) es una enfermedad rara y potencialmente letal caracterizada por la infiltración de tejido fibrótico en el endocardio y miocardio. Es más comúnmente observada en regiones tropicales y subtropicales, y se cree que es causada por la combinación de factores genéticos y ambientales. Su patogénesis aún no está del todo clara, pero es posible que esté relacionada con una respuesta inmune anormal ante múltiples estímulos, incluyendo infecciones virales, parásitos, trastornos hematológicos y deficiencias alimentarias. Esta condición es una de las principales manifestaciones de los síndromes hipereosinofílicos (SHE). Pese a los avances en el manejo, el pronóstico de los pacientes con FEM persiste siendo pobre, con un alto riesgo de recurrencia y de progresión hacia insuficiencia cardiaca. A continuación, presentaremos el caso de un paciente joven con un cuadro de insuficiencia cardiaca de predominio derecho en el contexto de un síndrome hipereosinofílico de base, en quien se establece el diagnóstico de FEM según lo documentado en las imágenes cardiacas.

Endomyocardial Fibrosis: Case Report and Literature Review. Endomyocardial fibrosis (EMF) is a rare and potentially life-threatening cardiac disorder characterized by the infiltration of fibrotic tissue into the endocardium and myocardium of the heart. It is most seen in tropical and subtropical regions, and it is believed to be caused by a combination of environmental and genetic factors. The exact pathogenesis of EMF is not fully understood, but it is thought to be related to an abnormal immune response to various stimuli, including viral infections, parasites, and dietary deficiencies. This condition is one of the main manifestations of hypereosinophilic syndromes. Despite advances in treatment, the prognosis for patients with EMF remains poor, with a high risk of recurrence and progression to heart failure. We present a case of a young male patient with an episode of right heart failure in the context of a chronic hypereosinophilic syndrome in which the diagnosis of EMF is made with the aid of cardiovascular imaging.

Mepolizumab in hypereosinophilic syndrome: a systematic review and meta-analysis

We aimed to evaluate the efficacy and safety of mepolizumab (MEP) in the management of hypereosinophilic syndrome (HES). A systematic search was performed, and articles published until March 2021 were analyzed. The primary efficacy results evaluated were hospitalization rate related to HES, morbidity (new or worsening), relapses/failure, treatment-related adverse effects, prednisone dosage ≤10 mg/day for ≥8 weeks, and eosinophil count <600/μL for ≥8 weeks. A meta-analysis was conducted, when appropriate. Three randomized controlled trials (RCTs), with a total of 255 patients, were included. The studies contemplated the use of MEP 300 mg/SC or 750 mg/IV. According to the evaluation of the proposed outcomes, when relapse rates/therapeutic failures were assessed, there was a 26% reduction with MEP 300 mg/SC (RD=-0.26; 95% CI: -0.44 to -0.08; p=0.04) and 48% reduction with MEP 750 mg/IV (RD=-0.48; 95% CI: -0.67, -0.30; p<0.00001). For the outcomes, prednisone dosage ≤10 mg/day for ≥8 weeks was 48% (RD=0.48; 95% CI: 0.35 to 0.62; p<0.00001), and the eosinophil count <600/μL for ≥8 weeks was 51% (RD=0.51; 95% CI: 0.38 to 0.63; p<0.00001), both showed a reduction with MEP 300 mg/IV and 750 mg/IV. No statistically significant differences in treatment-related adverse effects outcomes were observed for either dosage (RD=0.09; 95% CI: -0.05 to 0.24; p=0.20; RD=0.09; 95% CI: -0.11 to 0.29; p=0.39). Despite the positive effects observed for the studied outcomes, the exact significance remains unclear.

A case of IgG4-related disease associated with psoriasis-like skin rash and hypereosinophilic syndrome

Immunoglobulin (Ig) G4-related disease (IgG4-RD) is newly recognized immune-mediated and fibroinflammatory conditions with various organ involvements. Any organs can be involved, but the pancreas, salivary gland, lymph nodes, and orbit are known to be commonly involved organs. A 54-year-old man presented with complaint of psoriasis like skin rash developed 4 years prior to admission. Although he had been treated for skin rash, the extent of skin lesions increased as well as hypereosinophilia, and multiple lymphadenopathies were newly developed. The patient was diagnosed with IgG4-RD by serum IgG4 levels and histologic examination of the inguinal lymph node. One month after treatment with steroid and azathioprine, his skin rash and lymphadenopathies resolved with improvement and eosinophil count was within the normal range. We herein report a case of a IgG4-RD patient associated with psoriasis-like skin rash and hypereosinophilic syndrome.

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.

Eosinophilic Annular Erythema in a Patient with Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) / 대한피부과학회지

No abstract available.

Echocardiographic features of cardiac damage caused by hypereosinophilic syndrome / 西安交通大学学报(医学版)

Objective: Hypereosinophilic syndrome (HES) is a hematological disorder that may involve multiple organs, especially the heart as the targeting organ, and is the most important cause of death among patients. This paper summarizes the clinical and echocardiographic results of patients diagnosed with HES so as to provide an accurate and feasible method for early detection and timely treatment of the disease. Methods: We retrospectively analyzed the clinical data of five HES patients who came to the Department of Cardiology, Xi'an Jiaotong University, from 2004 to 2014 with the purpose of finding out the common and specific manifestations in transthoracic echo imaging. Results: There were five patients diagnosed with HES, with four cases of idiopathic HES and one of secondary HES after parasitic infection. Atrial enlargement was detected in the five patients, including four cases with double atria expansion and two cases with ventricular dilatation. Endocardial thickening was observed in all the patients with thrombosis, including four cases of thrombus detected in the double chambers' apex of and one case of thrombus only in the left ventricular apex. Changes of the valves and the pericardium were also shown in the transthoracic echocardiographic images and might be the characteristic features. Conclusion: Cardiac damage caused by HES is the main cause of death in the patients. Echocardiography can accurately show characteristic changes and provide help for early detection and correct treatment.

Acute myeloid leukemia with FIP1-like-1-platelet-derived growth factor receptor α fusion gene positive progressed from idiopathic hypereosinophilic syndrome: report of one case and review of literature / 白血病·淋巴瘤

Objective@#To improve the understanding of idiopathic hypereosinophilic syndrome (HES) and to be aware of its potential of transforming to acute myeloid leukemia (AML).@*Methods@#The clinical data of one patient diagnosed with HES progressed to AML in the 923rd Hospital of the People's Liberation Army Joint Service Support Force were analyzed, and relevant literatures were reviewed.@*Results@#The patient was diagnosed with idiopathic HES that progressed to AML with FIP1-like-1-platelet-derived growth factor receptor α (FIP1L1-PDGFRA) after 2 years. The patients achieved complete remission after the treatment of chemotherapy combined with tyrosine kinase inhibitor and then received hematopoietic stem cell transplantation. The patient had more than 1 year disease-free survival until the deadline.@*Conclusion@#Idiopathic HES has the potential to transform to AML with FIP1L1-PDGFRA fusion gene positive, therefore regular follow-up should be emphasized.

Acute myeloid leukemia with FIP1‐like‐1‐platelet‐derived growth factor receptor α fusion gene positive progressed from idiopathic hypereosinophilic syndrome: report of one case and review of literature / 白血病·淋巴瘤

Objective To improve the understanding of idiopathic hypereosinophilic syndrome (HES) and to be aware of its potential of transforming to acute myeloid leukemia (AML). Methods The clinical data of one patient diagnosed with HES progressed to AML in the 923rd Hospital of the Peopleˊs Liberation Army Joint Service Support Force were analyzed, and relevant literatures were reviewed. Results The patient was diagnosed with idiopathic HES that progressed to AML with FIP1-like-1-platelet-derived growth factor receptor α (FIP1L1-PDGFRA) after 2 years. The patients achieved complete remission after the treatment of chemotherapy combined with tyrosine kinase inhibitor and then received hematopoietic stem cell transplantation. The patient had more than 1 year disease-free survival until the deadline. Conclusion Idiopathic HES has the potential to transform to AML with FIP1L1-PDGFRA fusion gene positive, therefore regular follow-up should be emphasized.

Concurrent bilateral juvenile temporal arteritis and hypereosinophilic syndrome: a case report and review of the literature

Most of temporal arteritis occurs in the older patient over 50 years old, and the histopathologic finding shows a granulomatous inflammation, so this called giant cell arteritis. However, the young patients also present with a nodular lesion in their temple, and juvenile temporal arteritis (JTA) should be considered as one of the differential diagnosis, although it is very rare. For both diagnosis and treatment of JTA, excisional biopsy is essential. The pathologic finding of the temporal artery shows panarteritis with lymphoeosinophilic infiltrates, but no giant cell or granulomatous lesion. JTA is a localized disease with low level of systemic inflammatory marker, so the symptom is usually relieved by excision of affected lesion. Peripheral blood eosinophilia present in some cases of JTA, but its relation with clinical course and prognosis is not yet been known. Herein, we report the case of a 24-year-old man diagnosed with concurrent JTA and hypereosinophilic syndrome. We also reviewed the literature of JTA focusing on the impact of combined peripheral eosinophilia on the course of the disease. Combined peripheral eosinophilia may increase the risk of recurrence of JTA after local treatment such as excision only.

What Are the Clinical Features and Etiology of Eosinophilic Liver Infiltration?

BACKGROUND/AIMS: Although eosinophilic liver infiltration (ELI) is not rare, few data exist regarding its clinical characteristics and etiology. Therefore, we evaluated these aspects to better understand the clinical implications of this lesion type, which is reasonably common in Korea. METHODS: Patients suspected of having ELI, based on abdominal computed tomography results obtained between January 2010 and September 2017, were enrolled in this retrospective study. The presumptive etiologies of ELI were categorized as parasite infections, hypereosinophilic syndrome (HES), eosinophilic granulomatosis with polyangiitis (EGPA), malignancies, and unidentified. Clinical courses and treatment responses were also evaluated. RESULTS: The mean age of the enrolled patients (male, 237/328) was 62 years. Most patients (63%) were diagnosed incidentally and had peripheral eosinophilia (90%). Only 38% of the enrolled patients (n=126) underwent further evaluations to elucidate the etiology of the suspected ELI; 82 (25%) had parasite infections, 31 (9%) had HES, five (2%) had EGPA, and five (2%) had drug reactions in conjunction with eosinophilia and systemic symptoms. Almost half of the other enrolled patients had cancer. Radiologic resolution was achieved in 191 patients (61%; median time to radiologic resolution, 185 days). Resolution of peripheral eosinophilia was achieved in 220 patients (79%). In most cases, the course of ELI was benign. CONCLUSIONS: This large ELI study is unique in that the incidence rate, underlying diseases, and clinical courses were comprehensively evaluated. Clinicians should investigate the etiology of ELI, as several of the underlying diseases require intervention rather than observation.

A rare case of hypereosinophilic syndrome presenting with unilateral proptosis and torticollis

Hypereosinophilic syndrome (HES) is a spectrum of myeloproliferative disorder, which is characterized by persistent and marked blood eosinophilia and damage to multiple organs due to eosinophilic infiltration. Idiopathic HES is identified after ruling out all other causes of eosinophilia. Poor prognosis is usually associated with cardiac involvement and malignant transformation of blood cells. We report a rare case of HES in an 8-year-old boy who presented with unilateral proptosis and torticollis. The patient responded well to corticosteroid therapy with reduction of proptosis and torticollis and normalization of serum eosinophil count.

Endomiocardite de Loeffler ­ manifestação cardíaca da síndrome hipereosinofílica: relato de caso / Loeffler's endomyocarditis ­ cardiac manifestation of hypereosinophilic syndrome: case report'

Introdução: A síndrome hipereosinofílica é caracterizada por uma produção aumentada e contínua de eosinófilos e pode levar a lesões teciduais em múltiplos órgãos, como consequência da infiltração eosinofílica. Os pacientes apresentam eosinofilia absoluta no sangue periférico (> 1.500 eosinófilos/mm3) sem uma causa primária de eosinofilia. A manifestação cardíaca desta síndrome geralmente se apresenta como endomiocardite de Loeffler, que constitui uma miocardiopatia restritiva primária resultante da infiltração de eosinófilos no tecido cardíaco. Descrição do caso: Relatamos o caso raro de uma paciente de 64 anos com eosinofilia a esclarecer e comprometimento cardíaco, que teve o diagnóstico estabelecido a partir de exames de imagem. Comentários: Enfatizamos os aspectos clínicos e evolutivos, ressaltando as dificuldades diagnósticas e a importância da investigação de eosinofilias persistentes sem causa aparente, uma vez que o diagnóstico e tratamento precoce podem proporcionar melhores taxas de sobrevida e prognóstico nestes pacientes.

Introduction: The hypereosinophilic syndrome is characterized by an increased, continuous production of eosinophils, and it may lead to tissue damage in multiple organs as a consequence of eosinophilic infiltration. Patients with this syndrome present absolute eosinophil count > 1,500 eosinophils/mm3 in the peripheral blood without a primary cause for eosinophilia. The cardiac manifestation of this syndrome usually presents as Loeffler's endomyocarditis, a primary restrictive cardiomyopathy resulting from the infiltration of eosinophils into cardiac tissue. Case description: We report the rare case of a 64-year-old woman with eosinophilia and cardiac involvement, who had the diagnosis established based on imaging tests. Comments: We emphasize the clinical and evolutionary aspects of the condition, highlighting the diagnostic difficulties and the importance of investigating persistent eosinophilia without an apparent cause, as early diagnosis and treatment can provide better survival rates and improved prognosis in these patients.

Case of Idiopathic Hypereosinophilic Syndrome with Articular Involvement

Idiopathic hypereosinophilic syndrome (IHES) is a rare disease that is characterized by otherwise unexplained persistent eosinophilia and organ damage caused by eosinophilic infiltration. Its manifestations are highly variable but clinically apparent arthritis is uncommonly observed. Although Korean cases of severe eosinophilia in patients with rheumatoid arthritis (RA) or IHES concurrent with RA have been published, there are no reports of IHES with joint involvement. This paper reports a case of IHES presenting with persistent peripheral eosinophilia, fever, skin rash, multiple lymphadenopathy, and polyarthritis, including the distal interphalangeal joints of the hands.

Case report of one child with idiopathic hypereosinophilic syndrome and literature review / 天津医药

@#Idiopathic hypereosinophilic syndrome (IHES) is a very rare disorder in children, characterized by increased eosinophils in bone marrow, peripheral blood and tissue, resulting in multiple organ damage. We report a case of an 8-yearold girl with IHES, whose initial complaints were diarrhea and abdominal distension. Image examinations indicated that the digestive tract, lung and urinary bladder were all affected. Routine blood test showed that the eosinophilia was 20.64×109/L. Bone marrow smear showed that the mature eosinophilic granulocytes increased to 32%. The FIL1P1/PDGFRA, FGFR1 and IGH gene detection, parasites and antibody tests were all negative. The diagnosis of IHES was considered. The eosinophil decreased significantly and symptoms relieved after steroid treatment, though with recurrences, steroids were still sensitive. Then we reviewed the relevant literature and cases of children with IHES in China.

Gastroenteritis eosinofílica: A propósito de un caso y revisión de la literatura / Eosinophilic gastroenteritis: A case report and a review of the literature

La gastroenteritis eosinofílica es una enfermedad primaria rara caracterizada por abundante infiltrado eosinofílico en la mucosa intestinal. Se presenta con síntomas gastrointestinales diversos e inespecíficos de acuerdo a la profundidad de la afección de la mucosa, llegando a complicaciones severas. Se reporta el caso de una paciente de 52 años sin antecedentes previos con 8 meses de diarrea, dolor abdominal, ascitis y pérdida de peso. Tras realizarse los exámenes de rutina y descartarse todas las patologías más frecuentes, se llega al diagnóstico. La gastroenteritis eosinofílica requiere una alta sospecha diagnóstica y los síntomas crónicos generan gran impacto en el estado nutricional y calidad de vida del paciente, por lo cual instamos a los médicos a revisar el tema y considerarlo dentro del diagnóstico diferencial de diarrea y dolor abdominal crónicos no específicos.

Eosinophilic gastroenteritis is a rare primary disease distinguished by abundant eosinophilic infiltration of the intestinal mucosa. It concerns variable and nonspecific gastrointestinal symptoms according to the depth of infiltration and may cause severe complications. We reported a case of a 52-year-old woman without prior diseases with 8-month history of diarrhea, abdominal pain, ascites and weight loss. After having performed routine tests and rolled out most common diseases, we get to the diagnosis. Eosinophilic gastroenteritis requires a high suspicion for the diagnosis; because of chronic symptoms, it shows a burden on the nutritional status and quality of life of patients. Therefore, it is imperative that physicians should review this topic and consider it within differential diagnosis of chronic diarrhea and abdominal pain

Nursing care of one child with hypereosinophilic syndrome presenting multiple foot wounds / 中华护理杂志

This article summarized nursing care and management of a child with hypereosinophilic syndrome presenting five foot wounds.Nursing key points included:wound care,pain management and comprehensive interventions.Debriding and selection of dressing were based on the wound bed and acceptance of the patient;medication and psychological intervention were applied to reduce pain;the patient and his family members were encouraged to be involved in the management of wound management.Patient adherence was improved and pain was greatly reduced.After dressing change for 21 times in 93d,five foot wounds were all healed.

Hypereosinophilic Syndrome Associated with the Onset of Rheumatoid Arthritis: A Case Report

Idiopathic hypereosinophilic syndrome (HES) is a disorder characterized by the sustained overproduction of eosinophils and multiple organ damage. Rheumatologic manifestations of HES are infrequent, but persistent eosinophilia is observed in approximately 10% to 40% of patients with rheumatoid arthritis (RA). This finding may be a result of the RA itself and is often associated with active disease and the presence of extra-articular features. We describe the case of a 48-year-old man affected by HES who subsequently developed RA. Both HES and RA responded rapidly to the corticosteroid and methotrexate therapy. In this patient, the initiation of RA and HES was related, suggesting a common pathogenetic link between these two diseases.

Myeloid and lymphoid neoplasm with eosinophilia and abnormalities of PDGFRB presenting as congestive heart failure and hypereosinophilia

Hypereosinophilic syndrome (HES) is a heterogeneous disorder characterized by persistent hypereosinophilia with the evidence of organ dysfunction caused by eosinophilic involvement. HES can be induced by various secondary causes, including helminthic infections, adverse drug reactions, and allergic diseases. Primary/clonal bone marrow disease, including genetic mutations in platelet driven growth factor receptor alpha (PDGFRA), platelet driven growth factor receptor beta (PDGFRB), and fibroblast growth factor receptor 1 (FGFR1) could be its causes. Although corticosteroids are the mainstay of therapy in confirmed HES, imatinib is considered a definitive treatment for HES with these mutations. However, there have been few reports about HES with these genetic mutations in Korea. Here, we report a patient who presented with sudden onset of congestive heart failure and hypereosinophilia, proved to have PDGFRB rearrangement, and was controlled successfully with imatinib after left ventricle thrombectomy.