hemosiderosis with acute, subacute, alveolar hemorrhage Poliangeíte microscópica é uma forma de vasculite sistêmica de pequenos vasos, associada aos. Idiopathic pulmonary hemosiderosis (IPH) is a rare disorder with unknown pathogenesis that usually presents in the first decade of life. dad sistémica, en la que los estudios serológicos no propor- cionan datos concluyentes, y en general en . hemosiderosis. Microscopic polyangiitis. Systemic.
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Especially the heart is frequently affected. In this report, we describe 2 patients with HES. MRI findings of muscle involvement in idiopathic hypereosinophilic syndrome. A year-old white man presented with fever, muscle pain, skin nodules and persistent hypereosinophilia over a period of 1 year. In addition, he had ventricular arrhythmias with episodes of tachycardia. Besides a lack of response to antiparasitic therapy, laboratory and pathological data excluded the diagnosis of trichinosis or any other parasitic infection.
In a muscle biopsy several eosinophilic perivascular and leucocytic intravascular infiltrates were found, indicative of muscle involvement by the disease. This is a report on the MRI findings of muscle involvement in idiopathic hypereosinophilic syndrome. Idiopathic hypereosinophilic syndrome associated with rheumatoid arthritis A case report. Directory of Open Access Journals Sweden.
Reports on rheumatologic manifestations in patients with HES are very rare. In the case we report a typical rheumatoid arthritis developed in a year-old woman with HES treated with glucocorticoids. Because of the marked glucocorticoids side effects shown by the patient cushingoid habitus, hyperglycemia, we stopped this treatment and replaced it at first by methotrexate and later by cyclosporin, both of them associated with sulfasalazine. These drugs revealed very efficacious both on articular pathology and on the clinical and laboratory manifestations of HES.
These data suggest that common pathogenetic mechanisms are likely acting in rheumatoid arthritis and idiopathic hypereosinophilic syndrome.
Idiopathic hypereosinophilic syndrome involving the liver: College of Medicine, Seoul Korea, Republic of. To correlate CT features with peripheral eosinophilia in patients with idiopathic hypereosinophilic syndrome involving the liver.
During the last three years, features of liver involvement in nine of 20 patients with idiopathic hypereosinophilic syndrome were evaluated on CT.
In seven cases, interval change in hepatic lesion and the percentage of eosinophils were reviewed on follow-up examination. On initial CT, varying low-density patterns were seen in the liver in all cases; hepatomegaly was seen in four cases, and hepatosplenomegaly in two.
On follow-up CT, seven patients showed a decrease in the percentage of eosinophils, and in six, improved intrahepatic low densities were seen. On CT, intrahepatic low densities were seen in patients with idiopathic hypereosinophilic syndromeand these were distributed more extensively when peripheral eosinophilia was more severe. With improvement in peripheral eosinophilia, the low densities also improved.
Pyoderma gangrenosum in association with microscopic colitis, idiopathic hypereosinophilic syndromeselective IgE deficiency and diabetes mellitus.
Pyoderma gangrenosum PG is a neutrophilic dermatosis of unknown aetiology. We report a year-old male patient with diabetes, who presented with a nonhealing ulcer on the left leg, pruritic hyperpigmented papules distributed over the trunk and limbs, and chronic diarrhoea. He had eosinophilia, low haemoglobin and serum IgE levels, and raised erythrocyte sedimentation rate. Histopathology of the leg ulcer was consistent with the diagnosis of PG, while the histology of the hyperpigmented papule revealed tissue eosinophilia.
Dexamethasone pulse therapy achieved resolution of the ulcer and reduction in the eosinophilia. Further evaluation for the persistent diarrhoea led to a diagnosis of lymphocytic colitis LCwhich responded to budesonide.
Clinical management of the hypereosinophilic syndromes. Hypereosinophilic syndromes HESs are rare disorders characterized by marked hypereosinophilia that is directly responsible for organ damage or dysfunction. Different pathogenic mechanisms have been discovered in patient subgroups leading to the characterization of myeloproliferative and lymphocytic disease variants.
The practical clinical approach of patients with the different HES variants is reviewed herein, focusing on specific diagnostic tools and therapeutic options. The specific role of therapeutic compounds that have become available more recently, namely, tyrosine kinase inhibitors and IL-5 antagonists, is discussed.
Pulmonary involvement of hypereosinophilic syndrome: Hypereosinophilic syndrome is a rare entity of eosinophilic lung disease characterized by idiopathic prolonged eosinophilia of marked degree and variable organ involvement.
We report HRCT findings of three patients with pulmonary involvement of hypereosinophilic syndrome diagnosed by clinical manifestation, bronchoalveolar lavage and transbronchial lung biopsy.
On HRCT, several small nodules were seen in both lungs, especially in peripheral lung areas of the three patients. One had nodules with ground-glass attenuation halo and also focal areas of ground-glass attenuation in this area. Hypereosinophilic syndrome with hepatic involvement in a young child.
Hypereosinophilic syndromewhose etiology in unknown, involves the infiltration of various organs by a large number of eosinophils. The sites of involvement are the heart, skin, lung, liver, nervous system, and gastrointestinal tract. The disorder occurs mostly in middle-aged men and is characterized by striking peripheral eosinophilia.
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There have been few reports of hypereosinophilic syndrome in patients younger than 15 years and the disease also shows a predilection for males. We report a case of hypereosinophilic syndrome with hepatic involvement in a month-old girl, and correlate the imaging features with the pathologic findings.
We describe a case of idiopathic hypereosinophilic syndrome manifested by an axonal sensoriomotor polyneuropathy ASMP and signals of myositis. Sistemixa 20 years old woman began with progressive gait impairment with drops and presented with subacute lower limb edema associated with paresthesis and cramps.
She showed hypotonia in the lower limbs, absence of knee and ankle jerks, steppage gait, and decreased sensation on both legs. Examinations of stools were negative. Electrodiagnostic studies showed axonal lesion in sensory and motor nerves. Muscle biopsy showed type 2 muscle fibers atrophy with discrete inflammatory cells, predominantly lymphocytic in perivascular and interstitial locations.
She was treated with prednisone and all the symptoms subsided after two months. Biventricular thrombus in hypereosinophilic syndrome presenting with shortness of breath. Full Text Available A 48 years old hemosiderosus presented to clinic with 12 months of low grade fever with shortness of breath which has progressively worsened with no associated weight loss, night sweats or loss of appetite. There was no prior history of chronic illness before the current illness.
Laboratory workup revealed a high white blood cell count with predominant eosinophils. Chest X-ray was normal. Transthoracic echocardiography and Cardiac Magnetic Resonance showed biventricular thrombi. On further extensive workup the findings were consistent with hypereosinophilic syndrome. The patient was started on oral steroids, hydroxyurea, imatanib mesylate and oral anticoagulation. The patient responded to the treatment with complete resolution hemosidrrosis his symptoms over the course of few months.
The repeat Echocardiogram after a year showed normal left ventricular systolic and diastolic function with complete resolution of biventricular thrombi.
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Hypereosinophilic syndromeThrombus. A Rare Manifestation of Hypereosinophilic Syndrome. Full Text Available Several causes of eosinophilic pleural effusions have been described with malignancy being the commonest cause. We report a case of a year-old male who presented with shortness of breath. He had bilateral pleural effusions, generalized lymphadenopathy, splenomegaly, and leukocytosis with marked peripheral blood eosinophilia.
Hypereosinophilic syndrome was diagnosed after other causes of eosinophilia were excluded. He continued to be dyspneic with persistent accumulation of eosinophilic pleural fluid, even after his peripheral eosinophil count had normalized in response to treatment. This patient represents a very unusual presentation of HES with dyspnea and pleural effusions and demonstrates that treatment based on response of peripheral eosinophil counts, as is currently recommended, may not always be clinically adequate.
A case is presented of a year-old man with a history of the hypereosinophilic syndromewho subsequently developed granulocytic sarcoma of the tibia which proved resistant to aggressive therapy. He ultimately developed acute myeloid leukemia which also was resistant to therapy.
The disease process was characterized ssitemica localised skeletal pain, the development of lytic lesions in several areas of the skeleton and progression to frank myeloid leukemia. Full Text Available Hypereosinophilic syndrome is a blood disorder characterized by the overproduction of eosinophils in the bone marrow with persistent peripheral eosinophilia, associated with organ damage by the release of eosinophilic mediators.
Few cases of HES presenting with gastritis or enteritis have been reported worldwide. To date, HES presenting with esophagus involvement has only been reported once.
Here, we present a year-old Hispanic female patient with history of HES presenting with complaints of dysphagia and generalized pruritus. Additionally, eosinophilia may be due to connective tissue disorders, infections and occasionally to hematopoietic malignancies or solid sistdmica.
The criteria established in the s, for the definition of idiopathic hypereosinophilic syndrome is today unsatisfactory to characterize all. Biomedical research is extremely productive so that each day new discoveries reveal novel mechanisms and molecular pathways of diseases. However, the number of innovative therapies approved each year by regulatory authorities is decreasing. Hemosidetosis causes of this “innovation breakdown” are multiple and should be explored both in academic institutions and in the industrial world.
New initiatives are underway in the Walloon Region to improve this situation, such as the setting up of the BioWin Health Cluster gathering pharmaceutical and biotechnology enterprises and university laboratories. This new vision of the partnership between academia and the hemosidfrosis sector should be based on strong fundamental research activities. We illustrate this concept by discussing recent advances in the diagnosis and treatment of the hypereosinophilic syndrome.
Hypereosinophilic syndomes HESs include potentially lethal multisystem disorders characterized by eosinophilic infiltration of a variable spectrum of target organs, predominantly the skin, heart, lungs, gastrointestinal tract, and nervous system.
Based on recent advances in molecular and genetic diagnostic techniques and increasing experience with differences in clinical features and prognosis, subtypes have been defined, including “myeloproliferative-HES “, “lymphocytic-HES”, “familial eosinophilia”, “overlap HES”, “undefined HES” “complex undefined HES”, “simple undefined HES”, “episodic undefined HES” and “eosinophil associated diseases” such as Churg-Strauss syndrome.
HES should be kept in mind in the differential diagnosis of eosinophilic lung sietemica especially in patients with peripheral eosinophilia and pulmonary infiltrates.
Corticosteroids represent an effective firstline approach to decreasing eosinophil counts in the majority of cases. Imatinib might be used for corticosteroid nonresponders. We herein report a patient with “complex undefined HES” who had disease resistant to corticosteroids, but who had a significant response after treatment with imatinib.
A patient with hypereosinophilic syndrome that manifested with acquired hemophilia and elevated IgG4: Hypereosinophilic syndrome can cause coagulation abnormalities. Among hypereosinophilic syndrome types, the lymphocytic variant lymphocytic hypereosinophilic syndrome is derived from a monoclonal proliferation of T lymphocytes. Here, we describe the case of a patient with lymphocytic hypereosinophilic syndrome who presented with a coagulation abnormality.
To the best of our knowledge, this is the first such report including a detailed clinical picture and temporal cytokine profile.
His eosinophilia first appeared five years earlier when he developed femoral artery occlusion.