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1/14. hypereosinophilic syndrome: progression of peripheral neuropathy despite controlled eosinophil levels.

    Idiopathic hypereosinophilic syndrome (HES) is a disorder of the hematopoietic system, characterized by persistent elevation in the total eosinophil count (> 1500/microliter) for over 6 months, associated with organ damage and no detectable underlying cause. Treatment is centered on the reduction of total circulating eosinophils, which generally leads to remission of symptoms. We report a 68-year-old female patient with HES and peripheral neuropathy, presenting with cutaneous lesions, mental changes, cardiac and pulmonary symptoms, followed by right foot drop and eventually paraparesis, which caused an inability to ambulate. Weakness progressed to include the upper extremities despite adequate control of eosinophilia by steroids. Worsening of the peripheral neuropathy can occur despite lowering of the eosinophil levels.
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2/14. Recurrent cholestasis and hypereosinophilia in a young female.

    A 22-year-old female presented, in December 1998, with asthenia, itching and hypereosinophilia. In January 1997, due to the same clinical picture, the patient had inappropriately been diagnosed elsewhere to have an "idiopathic eosinophilic syndrome" and complete remission was obtained after short-term steroid treatment. Upon admission, physical examination was negative and blood tests revealed absolute eosinophilia [42%, i.e., 3,800 of 9,600 white blood cells], aspartate aminotransferase 4 x upper limits of normal, alanine aminotransferase 5 x upper limits of normal and alkaline phosphatase 2 x upper limits of normal. Both liver biopsy and endoscopic retrograde cholangiopancreatography findings were totally consistent with primary sclerosing cholangitis, while all known causes of hypereosinophilia and alteration in liver function tests were carefully excluded. The clinical course was characterized by complete clinical and biochemical normalization in absence of any treatment and further follow-up was completely negative.
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3/14. Observations on edema formation and resolution in Gleich syndrome: essential role of the kidneys in effective arterial blood volume regulation.

    Gleich syndrome is clinically present with episodes of angioedema, hypereosinophilia, oliguria, and weight gain due to fluid retention which may be sudden and remarkable, sometimes increasing to 10-20% of the baseline weight. The purpose of this study was to evaluate body fluid regulation and hormonal responses during the episode of angioedema and during the recovery phase in a patient with Gleich syndrome. A 24-year-old male was referred to our hospital for further evaluation of recurrent attacks of swellings of face, upper arms, and legs, marked weight gain, and oliguria. On first admission, the patient was in a remission phase, and the initial physical examination showed no abnormalities. Underlying disorders causing edema, such as heart, kidney, and liver diseases, and the recognized causes for hypereosinophilia, such as allergy, parasites, and collagen diseases, were ruled out. After 2 months, since his course was monitored, the patient was hospitalized. During days 10-19, he developed pronounced nonpitting edema of face, upper arms, and legs. Constant leukocytosis and hypereosinophilia, oliguria, and marked weight gain were also noted. A clinical remission was observed without any medication: intensive diuresis, loss of weight, regression of edema, and decreased eosinophil and leukocyte counts within 2 weeks. Physiological mechanisms during edema and resolution are discussed. In conclusion, our patient represents a suitable model for the protection of effective arterial blood volume because of the absence of underlying disorders causing edema. The kidneys play an essential role in the effective arterial blood volume regulation.
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4/14. Idiopathic hypereosinophilic syndrome involving the middle ear: clinical case report and discussion.

    Multiple organ system involvement is seen in the idiopathic hypereosinophilic syndrome (HES). Cardiovascular, lung and upper airway, liver, spleen, skin and nervous systems are frequently involved. In this article, we describe two cases of HES involving the middle ear and lung. In both cases, the histological pictures showed an increase of eosinophils in the tissue of the tympanic cavity.
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5/14. Chronic eosinophilic leukaemia presenting with erythroderma, mild eosinophilia and hyper-IgE: clinical, immunological and cytogenetic features and therapeutic approach. A case report.

    A 23-year-old, white male metallurgist presented with pruritic erythematous maculo-papules over the trunk and upper limbs and 6 months later developed erythroderma, eosinophilia and multi-organ dysfunction. A diagnosis of chronic eosinophilic leukaemia was made on the basis of myeloproliferative involvement of both peripheral blood and bone marrow, associated with eosinophilic differentiation and a t(5;12)(q33;p13) translocation. The initial therapeutic approach was interferon alfa-2b plus cytosine arabinoside, for 13 months, followed by hydroxyurea plus vincristine. There was improvement of skin lesions, disappearance of eosinophilia and decrease of serum immunoglobulin e, towards normal values.
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6/14. 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.
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ranking = 52.958492250658
keywords = abdominal pain
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7/14. Acute cholecystitis and duodenitis associated with churg-strauss syndrome.

    We describe a patient with acute cholecystitis and duodenitis associated with churg-strauss syndrome. A 36-year-old male, who had been healthy, had abdominal pain following high fever. He had marked hypereosinophilia of 17,000/mm3. Radiographs of the chest disclosed a transient infiltrated lesion in the left lower lung. Ultrasonographic and gastroendoscopic examinations revealed acute cholecystitis and duodenitis, respectively. Endoscopic retrograde cholangiopancreatography demonstrated a filling defect suspecting aberrant ascariasis in the common bile duct. The patient suddenly developed distally dominant mononeuritis multiplex, especially in the upper limbs. Muscle biopsy revealed vasculitis of intramuscular arteries with infiltration of eosinophils. These findings fulfilled the diagnostic criteria of churg-strauss syndrome. Corticosteroid dramatically resolved the abdominal symptoms. cholecystectomy and removal of the foreign body were performed. Histological examinations revealed that necrosis of the gallbladder was caused by occlusion due to thrombosed arteries and that the foreign body in the common bile duct was an aggregate of necrotic epithelium of the bile duct wall surrounded by inflammatory cells. Although abdominal complaints rarely appeared as an initial symptom in the patients with churg-strauss syndrome, this syndrome should be taken into consideration for an accurate diagnosis when the patients with abdominal pain of unknown origin had eosinophilia, asthma, or allergic rhinitis.
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ranking = 111.10608398599
keywords = abdominal pain, chest, upper
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8/14. A rare case of idiopathic hypereosinophilic syndrome involving the oral cavity associated with the esophagus and gastrointestinal tract.

    We report the rare case of HES involving oral cavity associated with esophagus, and gastrointestinal tract, which we succeeded in diagnosing precisely through a biopsy specimen obtained from the lip. A 64-year-old man had dysphagia, swelling of the oral mucosa and the posterior cervical muscles, accompanied by an abdominal pain and diarrhea. Peripheral blood cell count showed marked eosinophilia. Computed tomography showed thickening of posterior wall of the pharynx, esophagus, and gastrointestinal tract. Histologic specimen obtained from the lower lip demonstrated a moderate infiltration of eosinophils. His clinical condition was improved by oral prednisolone therapy.
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ranking = 52.958492250658
keywords = abdominal pain
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9/14. brachial artery occlusion secondary to hypereosinophilic syndrome.

    hypereosinophilic syndrome (HES) is characterized by an overproduction of eosinophils that leads to organ damage. Although most cases of HES frequently affect the lungs, heart, and gastrointestinal tract, there are a few reported cases of peripheral vascular involvement. We report a case of a patient with a history of colonic HES who presented with idiopathic occlusion of the brachial artery. A 28-year-old woman with a recent history of eosinophilic colitis presented with a several-week history of left hand pain, pallor, and paresthesias. Her hand was cool, without palpable pulses. Her eosinophilia count was 38%. An arteriogram documented a left brachial artery occlusion and diffuse left arm vasospasm. A brachial-to-brachial bypass was performed. Postoperatively, there was extensive vasospasm of her distal upper extremity arteries, which was treated with calcium-channel blockers and steroids. Her symptoms resolved and she has been asymptomatic for 9 months. The segment of occluded artery was found to contain many eosinophils on histologic examination. HES of the arterial system is an exceedingly rare cause of occlusion. Our patient presented with eosinophilia, arterial vasospasm with subsequent occlusion, and the presence of eosinophilic infiltration on the pathologic specimen. These data, combined with the patient's previous history, demonstrate that the patient's occlusion may have been secondary to HES.
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10/14. hypereosinophilic syndrome accompanying gangrene of the toes with peripheral arterial occlusion--a case report.

    The authors herein report the case of a teenage boy who presented with peripheral arterial occlusion of both upper and lower extremities associated with hypereosinophilia. During a 10-year follow-up, corticosteroid therapy was continued for the treatment of hypereosinophilia. The patient underwent bilateral lumbar sympathectomies because of severe ischemia of the bilateral lower extremities with gangrene of the toes. Based on the progress of his disease over the past 10 years, he was suspected to have idiopathic hypereosinophilic syndrome (HES) accompanied by peripheral arterial obstruction. Idiopathic HES is a disease characterized by unexpected hypereosinophilia, which may lead to organ damage. This is a very rare case of peripheral arterial occlusion associated with idiopathic HIS.
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