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1.
Medicines (Basel) ; 11(6)2024 Jun 02.
Artigo em Inglês | MEDLINE | ID: mdl-38921600

RESUMO

BACKGROUND: Idiopathic hypereosinophilic syndrome (IHES) is a disorder characterized by abnormal and persistent peripheral blood hypereosinophilia (eosinophil count ≥ 1.5 × 109/L and ≥10% eosinophils) with duration ≥ 6 months, associated organ damage, and/or dysfunction attributable to tissue eosinophilic infiltrate of unknown cause. IHES affects different organs such as the heart, lungs, nervous system, and skin, with renal involvement being rare in this condition. CASE PRESENTATION: We present a case of a young patient with IHES and immune complex-mediated membranoproliferative glomerulonephritis with nephrotic syndrome, as a rare renal manifestation. We discuss the clinical, analytical, and histopathologic renal and hematologic features, comparing them with other reported cases in the literature.

2.
Heliyon ; 10(5): e26885, 2024 Mar 15.
Artigo em Inglês | MEDLINE | ID: mdl-38468951

RESUMO

Eosinophilic gastroenteritis (EGE) is a rare disease which mainly consists of an abnormal eosinophile infiltration of the gastrointestinal tract. It's classified according to its location: eosinophilic esophagitis, eosinophilic gastritis, eosinophilic enteritis (including duodenum, jejunum and/or ileum) and eosinophilic colitis and degree of infiltration (mucosal, muscular, serosal). Depending on eosinophile concentration, type of EGE and the patient's condition it may manifest with different clinical presentations such as functional dyspepsia, abdominal pain, irritability, hypoproteinemia, diarrhea, anemia, among others. Few research has been done on such an uncommon pathology to the extent that treatment evidence is mostly limited to small case series. This case study reports an infrequent presentation of EGE in the small and large intestine as an undifferentiated gastrointestinal disease and successful corticoid management given to the patient in order to further broaden knowledge on this subject and facilitate an established clinical conduct for the treating physician.

4.
Case Rep Gastroenterol ; 17(1): 41, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36742098

RESUMO

Hypereosinophilic syndrome (HES) is a rare condition characterized by hypereosinophilia in peripheral blood or tissue infiltrate and organ damage. HES has been associated with several diseases, including inflammatory bowel diseases (IBDs), especially ulcerative colitis (UC). In this report, we describe a case of a UC and primary sclerosing cholangitis patient who was diagnosed with HES and severe cardiovascular and neurological injury. During hospitalization, an extensive diagnostic workup was performed and secondary causes of hypereosinophilia were ruled out. The patient was treated with glucocorticoids and full anticoagulation with significant clinical improvement and a marked reduction in the eosinophil count. In the literature, hypereosinophilia in the IBD population has been related to the severity of the disease and worse prognosis. The high index of clinical suspicion and the accurate diagnosis of HES are essential to avoid delay in therapy and prevent complications.

5.
Rev. costarric. cardiol ; 24(2)dic. 2022.
Artigo em Espanhol | LILACS, SaludCR | ID: biblio-1431794

RESUMO

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.


Assuntos
Humanos , Masculino , Adulto , Fibrose Endomiocárdica/diagnóstico por imagem , Insuficiência Cardíaca , Costa Rica
6.
Front Immunol ; 13: 938691, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35874692

RESUMO

Eosinophilic diseases, also termed eosinophil-associated diseases (EADs), are characterized by eosinophil-rich inflammatory infiltrates and extensive eosinophil degranulation with clinically relevant organ pathology. Recent evidence shows that eosinophil cytolytic degranulation, that is, the release of intact, membrane-delimited granules that arises from the eosinophil cytolysis, occurs mainly through ETosis, meaning death with a cytolytic profile and extrusion of nucleus-originated DNA extracellular traps (ETs). The ultrastructural features of eosinophil ETosis (EETosis) have been studied mostly in vitro after stimulation, but are still poorly understood in vivo. Here, we investigated in detail, by transmission electron microscopy (TEM), the ultrastructure of EETosis in selected human EADs affecting several tissues and organ systems. Biopsies of patients diagnosed with eosinophilic chronic rhinosinusitis/ECRS (frontal sinus), ulcerative colitis/UC (intestine), and hypereosinophilic syndrome/HES (skin) were processed for conventional TEM. First, we found that a large proportion of tissue-infiltrated eosinophils in all diseases (~45-65% of all eosinophils) were undergoing cytolysis with release of free extracellular granules (FEGs). Second, we compared the morphology of tissue inflammatory eosinophils with that shown by in vitro ETosis-stimulated eosinophils. By applying single-cell imaging analysis, we sought typical early and late EETosis events: chromatin decondensation; nuclear delobulation and rounding; expanded nuclear area; nuclear envelope alterations and disruption; and extracellular decondensed chromatin spread as ETs. We detected that 53% (ECRS), 37% (UC), and 82% (HES) of all tissue cytolytic eosinophils had ultrastructural features of ETosis in different degrees. Eosinophils in early ETosis significantly increased their nuclear area compared to non-cytolytic eosinophils due to excessive chromatin decondensation and expansion observed before nuclear envelope disruption. ETosis led not only to the deposition of intact granules, but also to the release of eosinophil sombrero vesicles (EoSVs) and Charcot-Leyden crystals (CLCs). Free intact EoSVs and CLCs were associated with FEGs and extracellular DNA nets. Interestingly, not all cytolytic eosinophils in the same microenvironment exhibited ultrastructure of ETosis, thus indicating that different populations of eosinophils might be selectively activated into this pathway. Altogether, our findings captured an ultrastructural signature of EETosis in vivo in prototypic EADs highlighting the importance of this event as a form of eosinophil degranulation and release of inflammatory markers (EoSVs and CLCs).


Assuntos
Eosinófilos , Síndrome Hipereosinofílica , Cromatina/metabolismo , DNA/metabolismo , Eosinófilos/metabolismo , Humanos , Síndrome Hipereosinofílica/patologia , Microscopia Eletrônica de Transmissão
7.
Clinics ; Clinics;76: e3271, 2021. tab, graf
Artigo em Inglês | LILACS | ID: biblio-1339708

RESUMO

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.


Assuntos
Humanos , Síndrome Hipereosinofílica/tratamento farmacológico , Anticorpos Monoclonais Humanizados/uso terapêutico , Prednisona/uso terapêutico , Contagem de Leucócitos
8.
Bol. méd. Hosp. Infant. Méx ; 76(3): 134-137, may.-jun. 2019. graf
Artigo em Inglês | LILACS | ID: biblio-1038898

RESUMO

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.


Assuntos
Humanos , Lactente , Masculino , Síndrome Hipereosinofílica/diagnóstico , Tosse/etiologia , Febre/etiologia , Síndrome Hipereosinofílica/fisiopatologia
9.
Bol Med Hosp Infant Mex ; 76(3): 134-137, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31116715

RESUMO

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.


Assuntos
Tosse/etiologia , Febre/etiologia , Síndrome Hipereosinofílica/diagnóstico , Humanos , Síndrome Hipereosinofílica/fisiopatologia , Lactente , Masculino
10.
Mol Clin Oncol ; 9(1): 35-39, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-29977537

RESUMO

Hypereosinophilic syndrome is a rare, chronic hematological disease characterized by a persistently elevated eosinophil count exceeding 1.5×109/l, following the exclusion of other potential etiologies. The systemic involvement of the disease causes tissue damage through eosinophil infiltration, and may affect various organs; cardiac complications are observed in 50-60% of cases, which are predominately attributed to endomyocardial fibrosis. The treatment is based initially on determining the presence of the FIP1L1-PDGFRA fusion. Patients with positive results for this mutation tend to achieve a complete response with imatinib treatment, which is thus the first line of treatment for this condition. However, patients who are negative for this mutation initially undergo treatment with corticosteroids. This study reports the case of a male 53-year-old patient diagnosed with hypereosinophilic syndrome in 2012, with negative results for the FIP1L1-PDGFRA mutation, and persistently high eosinophil levels, despite receiving the second line of standard treatment for this condition with hydroxyurea, and having already used corticosteroids without success. At the time of admission, the patient presented with acute decompensated heart failure due to severe mitral regurgitation, without any evidence of prior myocardial fibrosis or restrictive cardiomyopathy, and without suggestion of an associated ventricular hypertrophy. This clinical presentation is uncommon, as valvular involvement usually appears in the third stage of the development of cardiac involvement, and is usually associated with fibrosis and thrombotic events. Alternative therapeutic possibilities were evaluated due to the significant progression of the disease, and it was decided to attempt the use of imatinib, despite its use being preferably recommended for FIPIL1-PDGFRA-positive patients. The patient exhibited an evident and immediate response to imatinib, with normalization of the eosinophil count within 24 h of the first dose, which was maintained for at least the next 19 months. This clinical presentation is uncommon, as patients negative for FIPIL1-PDGFRA fusion do not frequently respond to imatinib treatment, and symptomatic heart failure usually appears in the third stage of disease progression.

12.
Arq. Asma, Alerg. Imunol ; 2(1): 148-152, jan.mar.2018. ilus
Artigo em Português | LILACS | ID: biblio-1380775

RESUMO

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.


Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Cardiomiopatia Restritiva , Síndrome Hipereosinofílica , Terapêutica , Tecidos , Ultrassom , Imageamento por Ressonância Magnética , Ecocardiografia , Diagnóstico
13.
Echocardiography ; 34(7): 1050-1056, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28600838

RESUMO

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.


Assuntos
Valva Aórtica/diagnóstico por imagem , Ecocardiografia Tridimensional/métodos , Síndrome Hipereosinofílica/diagnóstico por imagem , Adulto , Anticoagulantes/uso terapêutico , Valva Aórtica/fisiopatologia , Ecocardiografia/métodos , Ecocardiografia Transesofagiana/métodos , Feminino , Humanos , Síndrome Hipereosinofílica/tratamento farmacológico , Síndrome Hipereosinofílica/fisiopatologia
14.
Rev. gastroenterol. Perú ; 37(2): 177-181, abr.-jun. 2017. ilus, tab
Artigo em Espanhol | LILACS | ID: biblio-991248

RESUMO

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


Assuntos
Feminino , Humanos , Pessoa de Meia-Idade , Enterite/diagnóstico , Eosinofilia/diagnóstico , Gastrite/diagnóstico
15.
Rev. am. med. respir ; 16(4): 383-387, dic. 2016. ilus
Artigo em Espanhol | LILACS | ID: biblio-843020

RESUMO

El Síndrome Hipereosinofílico (SHE) constituye un grupo heterogéneo de enfermedades, que se caracterizan por una marcada eosinofilia en sangre, mayor a 1500 cel/mm³ de más de seis meses de evolución, asociado a daño de múltiples órganos, por infiltración eosinofílica ó liberación de mediadores. La eosinofilia es un hallazgo común en la práctica clínica, pero cuando se encuentran valores elevados de eosinófilos constituye un desafío diagnóstico. Presentamos el caso clínico de una paciente con antecedente de angioedema facial recurrente que consultó por tos poco productiva, toracodinia y disnea. El laboratorio mostró insuficiencia respiratoria con eosinofilia severa. La radiografía de tórax confirmó infiltrados pulmonares y derrame pleural. La paciente fue sometida a varios estudios complementarios, que nos orientaron hacia el diagnóstico de Síndrome Hipereosinofílico Idiopático, debido a la ausencia de causas secundarias y al hallazgo de eosinófilos en varios tejidos como médula ósea, líquido pleural, y lavado broncoalveolar. Se observó buena respuesta al tratamiento con corticoides sistémicos, logrando remisión completa de la enfermedad.


Hypereosinophilic Syndrome (HES) is a heterogeneous group of diseases, which are characterized by a marked eosinophilia in blood, greater than 1500 cells / mm³ over six months duration, associated with damage to multiple organs, eosinophilic infiltration, or release mediators. Eosinophilia is a common finding in clinical practice, but when levels of eosinophils are elevated can be a diagnostic challenge. We report the case of a patient with a history of recurrent facial angioedema, she consulted for some productive cough, dyspnea and toracodinia. The laboratory showed severe respiratory failure with eosinophilia. Chest radiography confirmed pulmonary infiltrates and pleural effusion. The patient underwent several complementary studies that guided us to the diagnosis of Idiopathic Hypereosinophilic Syndrome due to absence of secondary causes and finding of eosinophils in various tissues such as bone marrow, pleural fluid, and bronchoalveolar lavage. Good response to treatment with systemic corticosteroids was observed, achieving complete remission of the disease.


Assuntos
Derrame Pleural , Síndrome Hipereosinofílica , Angioedema
16.
Rev. argent. dermatol ; Rev. argent. dermatol;97(4): 2-5, dic. 2016. ilus
Artigo em Espanhol | LILACS | ID: biblio-843097

RESUMO

El síndrome hipereosinofílico constituye un grupo de enfermedades, caracterizadas por sobreproducción de eosinófilos con el daño secundario de múltiples órganos, provocado por la infiltración eosinofílica y la liberación de mediadores de inflamación. Es infrecuente y su prevalencia desconocida. El principal factor pronóstico es el compromiso cardíaco.


The hypereosinofilic syndrome is a group of diseases characterized by overproduction of eosinophils with secondary multiple organ damage caused by the cells infiltration and release of mediators of inflammation. It is infrequent and its prevalence is unknown. The main prognostic factor is the cardiac involvement.

17.
Rev. colomb. gastroenterol ; 29(4): 419-423, oct.-dic. 2014. ilus
Artigo em Espanhol | LILACS | ID: lil-742633

RESUMO

Introducción: la gastroenteropatía eosinofílica es una enfermedad rara caracterizada por infiltración de eosinófilos en una o más capas a diferentes niveles del tracto grastrointestinal, siendo común en estómago y duodeno. Aunque en la mayoría de los casos se presenta con dolor abdominal, también puede aparecer vómito, diarrea, ascitis y pérdida de peso. Reporte de caso: hombre de 41 años con cuadro clínico de 9 meses de evolución de dolor abdominal súbito e intermitente, de predominio en mesogastrio irradiado a región lumbar, manejado con antiespasmódicos sin mejoría. Antecedentes de: infarto agudo de miocardio 2 años atrás con arterias coronarias sanas según angiografía, nefrectomía derecha hace 11 años por hidronefrosis, diagnóstico y tratamiento desde hace 3 años de anemia microcítica hipocrómica con hipereosinofilia, apendicitis aguda hace 7 meses con hallazgos en la patología de infiltrados eosinofílicos, y una biopsia de médula ósea que reveló eosinofilia. Al examen físico: signos vitales normales, ruidos intestinales aumentados en intensidad y frecuencia (40/min) y mesogastrio doloroso a la palpación; sin masas ni visceromegalias. Sus laboratorios revelaron anemia y eosinofilia de 16%. Las imágenes diagnósticas reportaron una esofagogastroileítis, con evidencia microscópica de infiltración eosinofila en todas las muestras, por lo cual se diagnosticó gastroenteropatía eosinofílica iniciando manejo con dieta hipoalergénica y prednisona. Discusión: hasta 25% de los pacientes con síndrome hipereosinofílico puede tener infiltración gastrointestinal, por lo que la gastroenteropatía eosinofílica debe sospecharse en todo paciente con dolor abdominal y eosinofilia periférica, no obstante, esta última no siempre está presente, siendo necesario un diagnóstico histopatológico.


Introduction: Eosinophilic gastroenteropathy is a rare disease characterized by infiltration of eosinophils into one or more layers in different parts of the gastrointestinal tract especially the stomach and the duodenum. Although in most cases it presents with abdominal pain, vomiting, diarrhea, ascites and weight loss may also appear. Case report: The patient was a 41 year old man with a clinical picture of 9 months of sudden, intermittent abdominal pain (predominantly mesogastric pain which radiated to the lumbar region). He had been treated with antispasmodics but had not improved. Eleven years earlier he had had a right nephrectomy because of hydronephrosis. Three years earlier he had been diagnosed and treated for hypochromic microcytic anemia with hypereosinophilia. Two years earlier he had had an acute myocardial infarct, although angiography showed healthy coronary arteries. Seven months earlier he developed acute appendicitis. The pathology report at that time showed eosinophilic infiltrates, and a bone marrow biopsy revealed eosinophilia. Physical examination showed normal vital signs, but his bowel sounds were more intense and frequent (40/min) than normal, and he suffered mesogastric pain on palpation. There were no masses or organomegaly. Laboratory tests revealed anemia and 16% eosinophilia. Diagnostic images showed esophageal and gastric ileitis with microscopic evidence of eosinophil infiltration in all samples. Eosinophilic gastroenteropathy was diagnosed, and the patient was started on a hypoallergenic diet and treated with prednisone. Up to 25% of patients with hypereosinophilic syndrome may have gastrointestinal infiltration. Eosinophilic gastroenteropathy should be suspected in any patient with abdominal pain and peripheral eosinophilia. However, peripheral eosinophilia is not always present, and histopathological diagnosis is necessary.


Assuntos
Humanos , Masculino , Adulto , Gastroenterite , Gastroenteropatias , Síndrome Hipereosinofílica
18.
Rev. méd. Chile ; 142(4): 516-520, abr. 2014. graf, tab
Artigo em Espanhol | LILACS | ID: lil-716224

RESUMO

We report a 58 year-old-man without comorbid conditions, with a history of two months of weight loss, malaise and headache. His initial laboratory analysis showed leukocytosis of 16,100/mL with 65% eosinophils and an absolute eosinophil count of 10,465/mL. Both bone marrow biopsy and aspirate showed infiltration by mature appearing eosinophils. Treatment was started with hydroxyurea, associated with prednisone without satisfactory decrease in the eosinophil count. Polymerase chain reaction showed the presence of the gene fusion product FIP1L1/PDGFRA. Imatinib therapy was initiated, resulting in a rapid and progressive reduction in the absolute eosinophil count, with normalization at the second week of treatment. The incidence of the myeloproliferative variant causing hypereosinophilic syndrome is rare. However, the dramatic response to imatinib emphasizes the need to study the presence of the fusion product FIP1L1/PDGFRA in all patients with eosinophilia of unknown etiology.


Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Antineoplásicos/uso terapêutico , Benzamidas/uso terapêutico , Síndrome Hipereosinofílica/tratamento farmacológico , Piperazinas/uso terapêutico , Pirimidinas/uso terapêutico , Resultado do Tratamento
19.
Acta méd. colomb ; 39(2): 174-184, abr.-jun. 2014. ilus, tab
Artigo em Espanhol | LILACS, COLNAL | ID: lil-720230

RESUMO

Propósito de la revisión: revisar la literatura actual sobre el síndrome hipereosinofílico y establecercuáles son las diferencias y semejanzas con el síndrome de Churg-Strauss. Fuente de datos: revisión estructurada de la literatura médica, en las bases de datos Pubmed, Medline y Cochrane. Selección de estudios: se limitó la búsqueda a los artículos de revisión, guías de manejo y meta-análisis, sin límites en idioma ni fecha de publicación, utilizando los términos MESH: hypereosinophilic síndrome, eosinophilic, Churg-Strauss sindrome. Se acoplaron a términos como: update, review, associations. Extracción de datos: se hizo la lectura del resumen de 232 trabajos de los cuales se seleccionaron 111 que eran representativos para el tema de esta revisión, después de una clasificación y ordenamiento, se procedió a la redacción del texto. Conclusiones: el síndrome hipereosinofílico comprende un grupo heterogéneo de enfermedades con presentaciones clínicas muy variadas, para algunos autores el síndrome de Churg-Strauss o más recientemente conocido como granulomatosis eosinofílica con poliangiítis hace parte de este gran síndrome pero para otros, deben considerarse como entidades aparte. (Acta Med Colomb 2014; 39: 174-184).


Purpose of review: to review the current literature on the hypereosinophilic syndrome and establish which are the differences and similarities with Churg-Strauss syndrome. Source of data: structured review of the medical literature in databases Pubmed, Medline and Cochrane. Study selection: the search was limited to review articles, management guidelines and meta-analysis with no limits on language or publication date, using the MESH terms: hypereosinophilic syndrome, eosinophilic, Churg-Strauss syndrome. These were coupled to terms like:update, review, association. Data extraction: reading of the summary of 232 studies was made. Of these, 111 that were representative for the subject of this review, were selected, and after an ordered classification system, the wording was performed. Conclusions: The hypereosinophilic syndrome comprises a heterogeneous group of diseases with varied clinical presentations; for some authors, Churg-Strauss, more recently known as eosinophilic granulomatosis with polyangiitis, is part of this great syndrome, but for others these should be considered as separate entities. (Acta Med Colomb 2014; 39: 174-184).


Assuntos
Humanos , Masculino , Feminino , Adulto , Granulomatose com Poliangiite , Síndrome Hipereosinofílica , Síndrome de Churg-Strauss , Doença , Eosinofilia
20.
Arch. med. interna (Montevideo) ; 35(1): 23-26, mar. 2013. ilus
Artigo em Espanhol | BVSNACUY | ID: bnu-17699

RESUMO

El síndrome hipereosinofílico constituye un grupo de enfermedades caracterizadas por sobreproducción de eosinófilos con el secundario daño de múltiples órganos provocado por la infiltración eosinofílica y la liberación de mediadores de inflamación. Es raro y su prevalencia desconocida. El principal factor pronóstico es el compromiso cardíaco.


Assuntos
Humanos , Feminino , Idoso , Síndrome Hipereosinofílica/complicações , Síndrome Hipereosinofílica/diagnóstico , Síndrome Hipereosinofílica/terapia , Diagnóstico Diferencial
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