• Title/Summary/Keyword: Hypereosinophilic syndrome, idiopathic

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Idiopathic Hypereosinophilic Syndrome Involving Thoracic Spine

  • Park, Chi-Young;Kim, Seok-Won
    • Journal of Korean Neurosurgical Society
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    • v.47 no.5
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    • pp.389-391
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    • 2010
  • Hypereosinophilic syndrome is a rare hematologic disorder with sustained eosinophilia. Peripheral blood eosinophilia without an underlying etiology and with organs dysfunction has been designated hypereosinophilic syndrome. We report a rare case of symptomatic spinal infiltration of eosinophils at osteolytic T5 in patient with idiopathic hypereosinophilic syndrome.

A Case of Idiopathic Hypereosinophilic Syndrome Presenting Acute Pulmonary Edema (급성 폐부종으로 발현된 특발성 과호산구성 증후군 1예)

  • Yu, Kyong-Sul;Kim, Yeon-Jae;Seo, Hyang-Eun;Yoon, Hye-Jin;Do, Yun-Kyung;Lee, Byung-Ki;Kim, Won-Ho
    • Tuberculosis and Respiratory Diseases
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    • v.52 no.2
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    • pp.166-173
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    • 2002
  • Transient peripheral eosinophilia occurs in several disorders, such as allergic diseases, cancer, and parasitic infections. However, in most cases, their presence is not accompanied by tissue destruction or organ dysfunction. In certain disease states, eosinophils can accumulate in any organ in the body and cause tissue destruction as a result of the eosinophil infiltration or the toxic effects of the degranulated proinflammatory products. Idiopathic hypereosinophilic syndrome is a rare disorder characterized by persistent eosinophilia of an unknown origin, usually associated with a dysfunction of organs such as the heart, lung, skin, and nervous system. Idiopathic hypereosinophilic syndrome usually has an indolent course over a period of several months. However, in some cases, they have grave symptoms if vital organs such as heart and lung are infiltrated. Here we report a case of idiopathic hypereosinophilic syndrome presenting acute pulmonary edema involving the heart, bone marrow, and lung with a review of the relevant literatures.

A Case of Idiopathic Hypereosinophilic Syndrome Associated with Pulmonary Infiltration (폐 침윤을 동반한 특발성 과호산구 증후군 1례)

  • Rhu, Hon-Mo;Kweon, Young-Soo;Chung, Jin-Hong;Lee, Kwan-Ho;Lee, Hyun-Woo;Kim, Dong-Sug;Lee, Sam-Beom
    • Journal of Yeungnam Medical Science
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    • v.11 no.2
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    • pp.375-380
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    • 1994
  • The idiopathic hypereosinophilic syndrome consists of peripheral blood eosinophilia of $1500/mm^3$ or more without a known cause, plus signs and symptoms of organ eosinophilia. The prognosis of HES without treatment is poor. However, about one third of the patients with this syndrome may respond to corticosteroid thrapy. Morever, the majority of the remainder may have a favorable response to hydroxyurea. We present here a case of hypereosinophilic syndrome without any identifiable causes, involving bone marrow, liver, lungs and cervical lymph node. We tried corticosteroid as a treatment but it showed no response. However the hydroxyurea showed good response.

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Cytologic Findings of Bronchoalveolar Lavage (기관지 폐포 세정액의 세포학적 소견)

  • Kwon, Kun-Young;Cho, Seung-Che;Park, Kwan-Kyu;Chang, Eun-Sook
    • The Korean Journal of Cytopathology
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    • v.1 no.2
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    • pp.129-138
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    • 1990
  • Bronchoalveolar lavage (BAL) has emerged as a useful technique for the study of pulmonary interstitial disorders. Several types of Information are provided by the evaluation of lavage fluid identification of cellular constituents helps to separate inflammatory process. Recently we have studied cellular constituents of BAL from three cases with histologically confirmed pulmonary sarcoidosis, idiopathic pulmonary fibrosis and hypereosinophilic syndrome. Pulmonary sarcoidosis showed a marked increase in lymphocytes, idiopathic pulmonary fibrosis revealed a predominance of neutrophils, and hypereosinophilic syndrome presented a marked increase in eosinophils in the lavage fluids.

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Dysfunction of the Prosthetic Aortic Valve in Idiopathic Hypereosinophilic Syndrome -A case report - (인공 대동맥판막 기능부전을 일으킨 특발성 과호산구 증후군 -1예 보고-)

  • Yoo, Dong-Gon;Sung, Kyu-Wan;Jung, Sang-Sig;Kang, Gil-Hyun;Kim, Chong-Wook;Park, Chong-Bin
    • Journal of Chest Surgery
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    • v.40 no.4 s.273
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    • pp.297-300
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    • 2007
  • Idiopathic hypereosinophilic syndrome is a rare systemic, leukoproliferative disorder characterized by eosinophilmediated tissue injury causing multiple organ failure, including the heart. Cardiac involvement occurs in more than 75% of patients with hypereosinophilic syndrome. Cardiac manifestations include subendocardial fibrosis, thrombus leading to peripheral emboli, restrictive cardiomyopathy, and valvular dysfunction. It is more common in men than in women (9 : 1), and trends to present between the ages of 20 and 50 years. Presentation in childhood is unusual. We report for the first time a case of a 58-year-old man with idiopathic hypereosinophilic syndrome manifested by prosthetic aortic valve dysfunction that was successfully treated by steroid and hydroxyurea therapy after surgical valvular replacement.

Eosinophil disorders (호산구이상증)

  • Kim, Sun Young
    • Clinical and Experimental Pediatrics
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    • v.52 no.6
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    • pp.643-648
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    • 2009
  • Blood eosinophilia can be classified as either familial or acquired. Familial eosinophilia is a rare autosomal dominant disorder characterized by a stable eosinophil count. Acquired eosinophilia is classified further into a primary or secondary phenomenon depending on whether eosinophils are considered integral to the underlying disease. Primary eosinophilia is considered clonal in the presence of either a cytogenetic abnormality or bone marrow histological evidence of classified hematologic malignancies. Causes of secondary eosinophilia include infections, allergic or immunologic disorders, and drugs. Idiopathic eosinophilia belongs to a category of primary eosinophilia, and this is a diagnosis of exclusion. Cases with eosinophilia that lack evidence of clonality may be diagnosed as idiopathic hypereosinophilic syndrome after all causes of reactive eosinophilia have been eliminated. Genetic mutations involving the platelet-derived growth receptor genes (PDGFRA and PDGFRB) have been pathogenetically linked to clonal eosinophilia, and their presence predicts the treatment response to imatinib. In this review, I will present a clinical summary of both familial and acquired eosinophilia with emphasis on recent developments in molecular pathogenesis and treatment.

A Case of Idiopathic Hypereosinophilic Syndrome Complicated with Pulmonary Thromboembolism and Disseminated Intravascular Coagulation (폐색전증과 파종성혈관내응고가 합병된 특발성 과호산구 증후군 1례)

  • Lee, Jong Sung;Lee, Hyun-Kyung;Lee, Sung Soon;Jin, Jae Yong;Lee, Young Min;Lee, Hyuk Pyo;Kim, Joo In;Ku, Bon Il;Choi, Soo Jeon;Yum, Ho-Kee
    • Tuberculosis and Respiratory Diseases
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    • v.57 no.6
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    • pp.573-578
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    • 2004
  • The idiopathic hypereosinophilic syndrome (HES) comprises a heterogeneous group of disorders with unknown pathogenesis characterized by persistent peripheral blood and bone marrow eosinophilia. And the eosinophil infiltrates of multiple organs in HES lead to severe organ dysfunction. The disseminated intravascular coagulation (DIC) is a rare complication of HES. We have experienced a case of HES complicated with DIC and pulmonary thromboembolism. After intravenous injection of methylprednisone, blood eosinophil count was normalized but DIC was persisted. With cortico steroid and cyclosporine therapy, the disease activity was favorably remitted.

A Case of Hypereosinophilic Syndrome Presenting with Bilateral Pleural Effusions & Recurrent Bilateral Pneumothoraces (양측성 흉막 삼출과 재발성 기흉으로 발현된 과호산구증후군 1예)

  • Shim, Jae Min;Moon, Jin Wook;Hwang, Sang Yun;Do, Mi Young;Park, Moo Suk;Chung, Jae Ho;Kim, Young Sam;Chang, Joon;Kim, Sung Kyu;Cho, Sang Ho;Kim, Se Kyu
    • Tuberculosis and Respiratory Diseases
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    • v.57 no.5
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    • pp.470-475
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    • 2004
  • Idiopathic hypereosinophilic syndrome (HES) is a disorder characterized by prolonged eosinophilia without an identifiable cause and eosinophil related tissue damage in multiple organs including heart, lung, skin, gastrointestinal tract, liver, and the nervous systems. Pulmonary involvement occurs in about 40% of HES cases, but pleural effusion due to pleuritis and bilateral pneumothoraces are very rare manifestations. We report a case of hypereosinophilic syndrome presented with bilateral pleural effusions and recurrent bilateral pneumothoraces in a 44 year-old male with brief review of the literature.

Progressive Lung Involvement during Steroid Therapy in Idiopathic Hypereosinophilic Syndrome (특발성 과호산구 증후군에서 스테로이드 투약 중 진행한 폐침윤 1례)

  • Park, Ji Young;Choi, Ju Young;Jung, Ji Min;Sim, Yun Su;Pyun, Gun Woo;Na, Youn Ju;Kang, Min Jung;Kang, In Sook;Lee, Si Nae;Kim, Yookyung;Jeong, Jee-Hyong;Lee, Jin Hwa;Cheon, Eun Mee;Chang, Jung Hyun
    • Tuberculosis and Respiratory Diseases
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    • v.59 no.1
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    • pp.97-103
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    • 2005
  • Hypereosinophilic syndrome (HES) is characterized by a sustained eosinophilia of $1,500/mm^3$ or more in the absence of any known causes or the signs and symptoms of organ involvement. We report a 64-year-old man with HES initially presenting with involvement of the liver and bone marrow. Despite controlling the eosinophilia by corticosteroid, he developed a cerebral infarction and later progressive interstitial pneumonia. Brain angiography revealed a severe stenosis of the proximal right internal carotid artery (ICA) and a complete obstruction of the intracranial ICA. An open lung biopsy revealed fibrosis and lymphoplasma cell infiltration without eosinophils, which were consistent with nonspecific interstitial pneumonia.