Hepatitis hipóxica en paciente con endomiocardiofibrosis / Hypoxic hepatitis in a patient with endomyocardial fibrosis

La endomiocardiofibrosis es una causa de miocardiopatía restrictiva frecuente en la región de África subsahariana, aunque poco frecuente en nuestra población. Posee estrecha relación con la presencia de hipereosinofilia en sangre y tiene alta morbimortalidad. La hepatitis hipóxica es una afección clínica con un patrón enzimático característico, muy prevalente en unidades de cuidados intensivos y elevada mortalidad. Se reconocen múltiples mecanismos fisiopatológicos, como la isquemia, la congestión venosa y la alteración en la utilización de oxígeno del hepatocito. Describimos el caso de u na paciente de 35 años, consumidora de cocaína, con diagnóstico de endomiocardiofibrosis secundario a síndrome hipereosinofílico idiopático que presentó shock cardiogénico y hepatitis hipóxica asociada. Evolucionó favorablemente con el tratamiento de sostén adecuado.

Endomyocardial fibrosis is a restrictive cardiomyopathy with high morbidity and mortality rates, prevalent in the sub-Saharan Africa region but infrequent in our population. It has a close relation with blood hypereosinophilia. Hypoxic hepatitis is frequently observed in intensive care units and its diagnosis is clinical. It shows a typical enzyme pattern with high mortality too. There are multiple mechanisms responsible for this condition, such as ischemia, passive congestion and dysoxia. We described the case of a 35 year-old cocaine addict woman diagnosed with endomyocardial fibrosis and hypereosinophilic syndrome who developed cardiogenic shock with hypoxic hepatitis. The patient evolved favorably with the appropriate treatment.

Case for diagnosis. Erythroderma as manifestation of hypereosinophilic syndrome

Abstract: Hypereosinophilic syndrome is defined as persistent eosinophilia (>1500/µL for more than six months) associated with organ involvement, excluding secondary causes. It is a rare, potentially lethal disease that should be considered in cutaneous conditions associated with hypereosinophilia. We report a case of erythroderma as a manifestation of hypereosinophilic syndrome. A 36-year-old male with no comorbidities presented progressive erythroderma, pruritus, peripheral neuropathy, and eosinophilia in the previous seven months. No mutations were found in FIP1L1/PDGFRA. Patient experienced rapid remission in response to oral prednisone and hydroxyurea. Cutaneous manifestations may be the only evidence of hypereosinophilic syndrome. Genotyping excludes myeloproliferative disease, thereby orienting treatment and prognosis.

Cardiogenic shock with hypereosinophilic syndrome.

We report a child with hypereosinophilic syndrome who presented with cardiogenic shock. In addition, she had skin and joint involvement. The clinical condition improved and eosinophil counts normalized with steroid therapy. However, the skin lesions and hypereosinophilia relapsed on stopping the steroids. The child was subsequently maintained in remission on low dose prednisolone.

Hypereosinophilic syndrome with isolated Loeffler's endocarditis: complete resolution with corticosteroids.

Hypereosinophilic syndrome (HES) is classically defined as prolonged, unexplained peripheral eosinophilia in a patient presenting with evidence of end-organ damage. The heart is involved in two forms; endomyocardial fibrosis (Davies disease) and eosinophilic endocarditis (Loffler's endocarditis). It was first reported in 1968 by Hard and Anderson. Chusid and co-workers formulated a definition with strict criteria for the diagnosis of HES as 1) peripheral blood eosinophilia more than 1500 cells/cu mm for at least six months duration 2)signs, symptoms of end-organ (heart, lungs, gastrointestinal tract, skin, bone-marrow, brain) involvement with eosinophil tissue infiltration/injury 3) exclusion of known secondary causes of eosinophilia. We report a case of hypereosinophilic syndrome with Loffler's endocarditis, in the absence of endomyocardial fibrosis. The patient presented with a eosinophilic vegetation over the posterior leaflet of the mitral valve. There was complete resolution of the vegetation after two months of corticosteroid therapy.

Acute coronary syndrome associated with hypereosinophilia.

Patients with hypereosinophilia often present with symptoms and signs of other systemic disease.We present the case of a 30-year-old man who had hypereosinophilia presenting as acute coronary syndrome, while having normal coronaries. The history, clinical examination and laboratory investigations were consistent with a diagnosis of tropical eosinophilia, which responded promptly to diethyl carbamazine.

Factor V Leiden mutation and deep venous thrombosis in a patient with hypereosinophilic syndrome

Idiopathic hypereosinophilic syndrome is a rare condition characterized by extremely high peripheral blood eosinophil counts. Patients with idiopathic hypereosinophilic syndrome are at increased risk for thrombosis. The coexistence of idiopathic hypereosinophilic syndrome with other thrombotic disease is rare. We present an additional case of idiopathic hypereosinophilic syndrome and factor V Leiden mutation, which lead to deep vein thrombosis

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.

Exantemas febriles no infecciosos / Non infectious febrile rash

Exantema es una erupción de la piel con lesiones de distinto tipo, configuración y disposición. El diagnóstico diferencial para pacientes febriles con exantema es extenso. Las posibles causas son enfermedades infecciosas, drogas, enfermedades dermatológicas, inmunológicas y/o neoplásicas. Una historia detallada y un examen físico cuidadoso pueden ser esenciales para hacer un diagnóstico correcto. La historia debe incluir el sitio de comienzo, porcentaje y dirección de extensión, presencia o ausencia de prurito y relación temporal con la fiebre. En este artículo se revisarán diagnósticos no infecciosos de exantemas febriles

A Case of Idiopathic Hypereosinophilic Syndrome with Hepatic Involvement in a 5-Year-Old Boy / 대한간학회지

Idiopathic hypereosinophilic syndrome is defined as the presence of prolonged eosinophilia without an identifiable underlying cause and with evidence of end-organ dysfunction. The organs involved are the heart, bone marrow, nervous system, lungs, liver, skin, and gastrointestinal tract. Hepatic involvement is found in about 30% of patients of idiopathic hypereosinophilic syndrome. It occurs rarely in infants and children. In this report, we experienced one case of idiopathic hypereosinophilic syndrome with hepatic involvement in a 5-year-old boy who complained of intermittent fever and right upper quadrant abdominal pain. An abdominal ultrasound examination revealed an ill-defined low-echoic lesion in the liver. Pathologic findings of a biopsy specimen clearly showed the infiltration of eosinophils in the liver. Laboratory data disclosed absolute eosinophilia. There was no evidence of allergic disease or parasitic infestation.

Successful Cyclophosphamide Therapy in Recurrent Eosinophilic Colitis Associated with Hypereosinophilic Syndrome

Eosinophilic colitis is a relatively rare complication of hypereosinophilic syndrome which is characterized by abdominal pain and bloody diarrhea and is usually treated with steroids and hydroxyurea. However, no standard regimen exists in cases of intractable disease despite several treatment attempts with Interferon- alpha, cyclosporin, etoposide, and vincristine, etc. We here report a case of a 43-year-old woman with recurrent eosinophilic colitis as a complication of hypereosinophilic syndrome who was successfully treated with cyclophosphamide.

Toxocariasis en adulto manifestada como síndrome hipereosinofílico con compromiso neurológico predominante: caso clínico / Toxocariasis manifesting as an hypereosinophilic syndrome with predominant neurological involvement: report of adult case

Hypereosinophilic syndrome is characterized by persistent hypereosinophilia and signs or symptoms due to organ involvement, specially nervous system, heart and skin. It can be primary or secondary to allergies, parasites or cancer. Toxocariasis is an uncommon parasitic disease in adults. There is a variant, called visceral larva migrans, that can involve different organs, and among those, the central nervous system. We report a 61 years old male, with a cerebrovascular disease. There were focalizing symptoms, the CAT scan showed multiple ischemic lesions and a peripheral eosinophilia of 12152 cells/mm3 was present. Anti toxocara IgG antibody titers were 1/1000. The patient was treated with albendazole for 14 days. After a 2 years follow up the patients is in good conditions and, for the first time, his eosinophil count is within normal limits

Fracturas lumbares en un paciente con síndrome hipereosinofílico idiopático / Lumbar fractures in a patient with idiopathic hypereosinophilic syndrome

El síndrome hipereosinofílico idiopático (SHI) es un trastorno hematológico poco frecuente que causa activación y degranulación de eosinófilos en diversos tejidos con el consecuente daño tisular y funcional. Los tejidos más afectados son: corazón, pulmón, piel, sistema nervioso, aparato digestivo y riñón. El ataque al hueso y específicamente a columna vertebral, es de extrema rareza. El objetivo del tratamiento es frenar la producción de eosinófilos y de su actividad lítica degranuladora mediante esteroides, citotóxicos, alfa interferón o trasplante de médula ósea. Se informa el caso de un hombre de 32 años con (SHI) con antecedente de púrpura en la infancia, cardiomiopatía dilatada, evento trombótico isquémico cerebral y lesiones en las vértebras lumbares 2 y 4, canal estrecho lumbar y paraparesia desproporcionada, manejado con artrodesis lumbar mediante tornillos transpendiculares y descompresión lumbar, con mejoría importante de la fuerza muscular que le permitió reinicial la deambulación por sí mismo. Los estudios de imagen de control a los seis meses de la cirugía, muestran adecuada alineación del segmento intervenido y no hay evidencia de progresión de la lesión hacia las vértebras adyacentes

Pulmonary involvement in idiopathic hypereosinophilic syndrome.

A case of idiopathic hypereosinophilic syndrome (HES) is presented. The patient had been symptomatic and had documented peripheral blood eosinophilia for 9 years. The patients having only pulmonary involvement, seem to have a good prognosis and hence must be considered as a separate subgroup of HES.