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.

Tropical Pulmonary Eosinophilia : Masquerading as Eosinophilic Leukaemia.

Benign conditions like Tropical Pulmonary Eosinophilia(TPE) can present with very high total count and Absolute Eosinophil Count (AEC) and can mimick malignancy. Diagnostic work up for TPE should be done in any patient presenting with pulmonary symptoms and eosinophilia. Though most case series on TPE report AEC in range of 3000 to upto 20,000, very rarely AEC can rise beyond 50,000. The following case is of TPE presenting with absolute eosinophil count of >70,000. Rapid response to Diethyl carbamazine is the rule in a confirmed case of TPE.

Síndrome hipereosinofílica idiopática: relato de caso e revisão de literatura / The idiopathic hypereosinophilic syndrome: case report and literature review

JUSTIFICATIVA E OBJETIVOS: A síndrome hipereosinofílica idiopática (SHI) é uma desordem mieloproliferativa rara, marcada pela contínua superprodução de eosinófilos, ocasionando lesões em órgãos específicos, incluindo o coração. O diagnóstico é definido pela combinação de eosinofilia prolongada inexplicada e evidência de envolvimento sistêmico. O objetivo deste estudo foi discutir um caso típico desta doença, enfatizando seus aspectos clínicos, evolutivos e terapêuticos com o intuito de divulgar e ressaltar a importância da investigação de eosinofilias persistentes sem causa aparente.RELATO DO CASO: Paciente do sexo masculino, 54 anos, com sintomas neurológicos agudos, associados à eosinofilia há mais de seis meses e cardiomiopatia por infiltração eosinofílica. Após o diagnóstico de SHI foi instituído tratamento com corticoides, com boa resposta.CONCLUSÃO: É importante alertar a população médica para a necessidade da investigação de eosinofilias persistentes. Tratando-se de SHI, o diagnóstico e a terapêutica precoce melhoram a sobrevida, qualidade de vida ou até mesmo podem promover remissão desta doença rara.(AU)

BACKGROUND AND OBJECTIVES: The idiopathic hypereosinophilic syndrome (IHS) is a rare mieloproliferative disorder, marked by a sustained overproduction of eosinophils and damage to specific organs, including the heart. The syndrome is defined by the combination of unexplained prolonged eosinophilia and evidence of organ involvement. The objective of this study was to discuss a typical case of IHS, emphasizing its clinical, evolutives and therapeutical aspects and focusing the importance of exclude this disease in patients with persistent eosinophilias without a clear cause.CASE REPORT: Male patient, 54 years old, with acute neurological symptoms, persistent eosinophilia for more than six months and cardiomiopathy secondary to eosinophilic infiltration. After the diagnosis of IHS, he was treated with corticosteroids, with a good response.CONCLUSION: It's important to alert the medical population about the evaluation of the causes of persistent eosinophilias. In the case of IHS, the early diagnosis and treatment improves the survival, quality of life or in some cases it can promote remission of this rare disease.(AU)

Complexo primário da paracoccidioidomicose e hipereosinofilia / Primary complex of paracoccidioidomycosis and hypereosinophilia

A infecção primária por Paracoccidioides brasiliensis é observada raramente. Um paciente masculino de 28 anos de idade apresentou febre, sintomas respiratórios e indisposição durante três meses. Exames radiológicos evidenciaram infiltrado pulmonar apical bilateral, pleurite à direita e linfadenomegalia hilar. O paciente apresentava leucocitose, intensa eosinofilia e títulos crescentes de anticorpos séricos anti-P. brasiliensis. Até onde sabemos, este é o primeiro relato do complexo primário da paracoccidioidomicose, associado com hipereosinofilia, apresentado por um adulto previamente hígido.

Primary infection with Paracoccidioides brasiliensis has rarely been observed. A 28-year-old male patient presented with a three-month history of fever, respiratory symptoms and malaise. Chest X-rays revealed bilateral apical infiltrates, right pleuritis and hilar lymphadenomegaly. The patient presented with leukocytosis, severe eosinophilia and increasing titers of anti-P. brasiliensis antibodies in serum. To our knowledge, this is the first report of the primary pulmonary lymph node complex of paracoccidioidomycosis accompanied by hypereosinophilia and affecting a previously healthy adult.

Chronic eosinophilic leukemia with a unique translocation.

We report a case of chronic eosinophilic leukemia in a 9 year old girl who presented with anemia, thrombocytopenia, leucocytosis (mostly dysplastic eosinophils), lymphadenopathy and hepatosplenomegaly. There was no increase in blasts but myelofibrosis was seen in the bone marrow. A previously unreported translocation 46,XX,t(1;4)(q24;q35), was found on cytogenetic analysis and involvement of the myocardium was also present. Shortly after commencing steroids, the family abandoned therapy.

Clinical applications of molecular haematology: an overview.

Haematological disorders are getting more and more molecularly defined. This is specially so in the field of haematological malignancies. The molecular understanding has helped in fine-tuning the diagnosis, prognosis and management. The tests are becoming widely available and they enjoy both sensitivity and specificity. Following is an overview of clinical applications of molecular haematology, especially in the field of chronic myeloid leukaemia, chronic eosinophilic leukaemia, bcr abl negative chronic myeloproliferative disorders and acute promyelocytic leukaemia.

Treatment of idiopathic hypereosinophilic syndrome with imatinib mesylate.

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 report a young patient with HES who developed stroke 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 rapidly. Our finding concurs with recent reports that imatinib mesylate may be a promising agent in the treatment of some cases of HES.

Hypereosinophilic syndrome.

Hypereosinophilic syndrome is a leukoproliferative disease characterised by sustained overproduction of eosinophils. The three diagnostic criteria for this disorder are (1) Eosinophilia of greater than 1500 cells/ml, persisting for longer than 6 months, (2) lack of another diagnosis to explain the eosinophilia and (3) signs and symptoms of organ involvement. We report a 15-year-old boy who was diagnosed as Hypereosinophilic syndrome based on these criteria.

El eosinofilo / Eosinofilo

los eosinófilos son una minoría de los leucocitos de sangre periférica y su presencia en los tejidos se limita principalmente a los sistemas gastrointestinal, respiratorio y genitourinario. Entre sus principales funciones se encuentran; la generación de mediadores inflamatorios, la defensa del huésped y el remodelamiento tisular. En ciertas entidades patológicas estas células pueden acumularse selectivamente en la sangre periférica o en algún tejido corporal, originando alteraciones importantes. Existen múltiples condiciones en las cuales se presenta eosinofilia, entre ellas se destacan especialmente ciertas patologías alérgicas, enfermedades malignas, el rechazo de trasplantes, la hipersensibilidad a medicamentos y las infecciones parasitarias. Establecer un diagnóstico acertado a cada paciente requiere de una detallada evaluación clínica y de la utilización de pruebas de laboratorio más específicas de acuerdo con las condiciones del paciente. Palabras clave: eosinófilo, alergia, asma, dermatitis, helmintiasis, enfermedad maligna. Vanegas-Arroyave N, Campuzano-Maya, G. El eosinófilo. Medicina & Laboratorio 2005; 11: 311-319.Módulo 4 (Hematología), número 8. Editora Médica Colombiana S.A., 2005

Sindrome de Loeffler: presentación de un caso / Loeffler syndrome: a case report

Presentamos el caso de un niño pre escolar procedente de Los Yungas Paceños, que ingresó al Hospital del Niño de la ciudad de La Paz, por disentería, desnutrición y antecedentes de eliminación de Ascaris por boca, nariz y recto. Recibió mebendazol para su enteroparasitosis, con franca mejoría de su cuadro enteral, permaneciendo hospitalizado para otros agentes de disentería y rehabilitación nutricional. A los siete días de internación presenta en forma súbita dificultad respiratoria, datos clínicos de pulmón sibilante (sibilancias, roncus y crépitos finos en ambos pulmones), marcada eosinofília periférica de 24% (recuento absoluto de eosinófilos 3.360) e imágenes radiográficas de tórax con infiltrado intersticial difuso, bilateral compatible con una neumonía difusa intersticial. Por proceder de una región con elevada incidencia de enteroparasitosis masiva, eliminación de nematodos, eosinofilia simultánea, compromiso pulmonar sibilante, intersticial y transitorio antes descrito; se considera el cuadro como síndrome de Loeffler, recibiendo tratamiento como tal, con mejoría clínica.

We present the case of a preschool child coming from the Yungas of La Paz which was admitted to the Hospital del Niño in La Paz with dysentery , malnutrition and a history of elimination of Ascaris through mouth, nose and rectum. The child received mebendazol against the enteroparasites, resulting in a clear improvement of the dysentery, while remaining hospitalized for treatment of other dysentery agents and nutritional rehabilitation. At seven days of his stay, the child suddenly presented breathing difficulty, clinical signs of a "sibilant lung" (crepitating, sibilant and roncus sounds in both lungs), marked peripheral eosinophilia of 24% ( eosinophil count 3,360 cells) and radiografíc images of the torax with diffuse bilateral interstitial infiltrations compatible with diffuse interstitial pneumonía . Since the child comes from a region with a high incidence of massive enteroparasitism, elimination of nematodes, simultaneous eosinophilia, sibilant lung, interstitial and temporal previously described, the disease is considered as Loeffler's Syndrome and treated accordingly with clinical improvement.

Neuropatia periférica e miosite na síndrome hipereosinofílica idiopática: relato de caso / Peripheral neuropathy and myositis in idiopathic hypereosinophilic syndrome: case report

Descrevemos um caso de síndrome hipereosinofílica idiopática, com manifestações clínicas de neuropatia periférica e sinais de miosite inflamatória. Trata-se de mulher de 20 anos de idade, que apresentou dificuldade progressiva para caminhar com quedas freqüentes e edema de membros inferiores até o nível do joelho, associado a parestesias e cãibras. O exame neurológico revelou hipotonia, arreflexia e redução da força e sensibilidade nos membros inferiores. O exame parasitológico de fezes foi negativo e o hemograma mostrou 24 por cento de eosinófilos (1848/mm ). Estudo eletrodiagnóstico mostrou comprometimento axonal sensitivo-motor nos nervos dos membros inferiores. A biópsia muscular mostrou discreta reação inflamatória perivascular e intersticial. Tratada com prednisona a paciente apresentou remissão dos sintomas em dois meses.

Idiopathic Hypereosinophilic Syndrome Presenting As a Single Mass in the Liver / 대한소화기학회지

Hypereosinophilic syndrome is characterized by persistent blood eosinophilia of 1,500/mm3 or more in the absence of known causes and multiorgan dysfunction by eosinophil-related tissue damage. In Korea, some cases of hypereosinophilic syndrome with hepatic involvement have been described with prolonged benign clinical courses. Most of them were diffuse or multifocal lesions in imaging studies, and any case presenting as a large single mass lesion has not been described. Herein we report a case of hypereosinophilic syndrome with hepatic involvement in a 48-year-old woman who presented with a giant single mass. By abdominal CT scan, a lobulated well-margined heterogenous mass lesion was detected in the left lateral segment of the liver. Liver biopsy revealed severe eosinophilic infiltration and centrilobular necrosis of hepatocytes. The lesion completely disappeared after steroid administration for eleven months.

Childhood idiopathic hypereosinophilic syndrome: report of a case.

We described a 15-year-old girl who presented with persistent fever, bilateral flank pain, and worsening dyspnea. The peripheral blood cell count showed remarkable eosinophilia at the time of admission. Severe pleural effusion with eosinophilic Infiltrations as well as pericardial effusion were noted thereafter. Bone marrow examination disclosed markedly increased eosinophils. Bilateral ectasia of the renal pelvis was found in an ultrasonographic study of the kidneys. Spiking fever and progressive shortness of breath persisted despite treatment with empiric antibiotics for infection. Based on the clinical course and histological findings, a tentative diagnosis of idiopathic HES was made. After treatment with oral prednisolone daily (1 mg/kg/day) for one week, there was a rapid improvement in her clinical condition. She was discharged a few days later and the steroids were withdrawn gradually when she was asymptomatic. The absolute eosinophil count (AEC) was monitored during follow-up. At 3 weeks, the AEC had fallen from 8,060/mm3 to 4,792/mm3 and it further fell to 1,591/mm3 at 5 months, and to 855/mm3 at 8 months during follow-up. There is no evidence of any other organ involvement until now. The clinical manifestations, diagnosis and management of idiopathic HES in children are also reviewed.

Síndrome hipereosinofílico: una causa rara de insuficiencia mitral grave en pediatría / Idiopathic hypereosinophilic syndrome: a rare cause of mitral valve failure in children

Introducción. El síndrome hipereosinofílico (SHE) es un desorden leucoproliferativo caracterizado por sobreproducción sostenida de eosinófilos sin causa definida. Es característico de la enfermedad la afección de diferentes órganos incluyendo el corazón. Es una entidad rara en adultos y aún más en edad pediátrica.Caso clínico. Masculino de 6 años de edad con SHE y cardiopatía adquirida caracterizada por insuficiencia mitral grave secundaria a fibrosis de la valva posterior. El diagnóstico se realizó por ecocardiografía y se corroboró por biopsia miocárdica. En espera de recambio de válvula mitral el paciente falleció secundario a insuficiencia cardiaca grave. Conclusión. En este paciente el ecocardiograma permitió el adecuado diagnóstico de la lesión cardiaca, siendo la imagen muy característica, que por experiencia no se ha visto en otros tipos de miocardiopatías. Dada la poca frecuencia de esta entidad la biopsia miocárdica sigue estando indicada para confirmar y estadificar la lesión.

Síndrome hipereosinofílica idiopática / Idiopatc hypereosinophilic syndrome

O caso clínico a seguir apresentado expressa, como evidência principal de sua evolução, hipereosinofilia de grandes proporções. Apesar da busca incessante de sua causa por intermédio de propedêutica intervencionista, observação clínica prolongada e uso de terapêutica de prova, não se obteve sucesso diagnóstico. Revela o limite no espaço e tempo da possibilidade diagnóstica, o flagrante reconhecimento de que, apesar da grande evolução técnica, a medicina continua desafiando a lógica, o raciocínio e a sensibilidade humana.

This article shows the clinical case of idiopatic hipereosinophilic syndrome. Although the continuous search through aggressive propedeutic, prolonged clinical observation, use or proof therapy, the successful diagnosis was not reached. The challenge of the exact diagnosis is the great oppression of the clinical practice. The unknow should not immobilize the medical judgement.

Excellent response to interferon therapy in a patient with hypereosinophilic syndrome and elevated serum immunoglobulin E levels

The hypereosinophilic syndrome (HES) is a heterogeneous disease characterized by sustained eosinophilia for a period of at least six months with evidence of organ involvement. Its manifestations range from a benign disorder not requiring any therapy to an aggressive, malignant variety refractory to common treatments. Diverse therapies have been used, including steroids, hydroxyurea, and chemotherapy, with variable responses. Recently alpha-interferon therapy has been shown effective in this disorder. Of the various prognostic factors, elevated serum immunoglobulin E (IgE) levels is considered among the most favorable, with most patients presenting with a [quot ]benign[quot ] disorder, not requiring therapy. We describe a patient presenting with an aggressive variant of HES despite having elevated IgE levels. The patient had a dramatic and lasting response to alpha-interferon

Leucemia Eosinofilica: reporte de un caso clínico / Eosinophilic leukemia: on pourpose of a case

Se describe un caso de leucemia eosinofilica en niñade 11 años sin alteraciones croosómicas, en fase de crisis blástica. Con cuadro clínico de compromiso gastrointestinal cutáneo, asociado a 21216 eosinófilos/mL en sangre periférica en un infiltrado del 60porciento en médula ósea.