• 제목/요약/키워드: Langerhans cell Histiocytosis

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국내 폐 랑게르한스세포 조직구증(Pulmonary Langerhans Cell Histiocytosis)의 임상양상 (Clinical Features of Pulmonary Langerhans Cell Histiocytosis in Korea)

  • 김철;정성환;심재정;차승익;손춘희;정만표;박혜윤;김영환;박종선;어수택;박춘식;김동순;조경욱;송진우;제갈양진;박무석;박병훈;이진화;허진원;염호기;이홍렬;박용범
    • Tuberculosis and Respiratory Diseases
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    • 제66권2호
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    • pp.98-103
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    • 2009
  • 연구배경: 폐 랑게르한스세포 조직구증(pulmonary Lan gerhans cell histiocytosis, PLCH)은 랑게르한스세포의 증식으로 형성된 육아종이 여러 장기에 침범하여 발생하는 질환이다. 주로 폐에 침범하며, 무증상에서부터 호흡곤란등의 다양한 임상증상을 가진다. 국외뿐만 아니고 국내에서도 빈도가 적은 것으로 알려져 있어, 다기관에서 국내 증례를 모아 그 임상양상을 알아보고자 하였다. 방 법: 부위에 관계없이 조직학적으로 진단되고 폐의 병변이 있는 PLCH 환자의 임상증상을 ILD 연구회를 통하여 후향적으로 조사하였다. 결 과: 수집된 증례는 총 56명으로 남자 48명, 여자 8명이었고, 중간나이는 43세(18-67세)였다. 진단 당시 흡연자와 과거 흡연자는 79%로 흡연과 관계가 있었다. 가장 흔한 증상은 기침(39%)이었고, 이어 호흡곤란(38%), 객담(20%), 흉통(20%) 순이었다. 기흉은 16명의 환자에서 관찰되었다. 폐기능 검사에서 정상은 26명(61%), 제한성 7명(16%), 혼합형 7명(16%), 폐쇄성은 3명(7%)에서 관찰 되었다. 방사선 소견은 대부분의 환자에서 병변이 중엽과 상엽에 있었으며, 결절과 낭종이 같이 있는 경우가 59%로 가장 많았다. 결 론: 이 연구는 PLCH 환자에 대한 국가적 그리고 장기적 추적 연구의 필요성을 시사한다.

Pineal and Suprasellar Germinoma Cooccurence with Vertebra Plana: A Case Report

  • Toosi, Farrokh Seilanian;Aminzadeh, Behzad;Rad, Mohammad Faraji;Nekooei, Sirous;Nahidi, Mahsa;Keykhosravi, Ehsan
    • Brain Tumor Research and Treatment
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    • 제6권2호
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    • pp.73-77
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    • 2018
  • Germinoma is the most common type of intracranial germ cell tumors (GCTs). Pineal gland and suprasellar region are the most frequent sites of central nervous system (CNS) involvement. Intracranial masses caused by Langerhans cell histiocytosis (LCH) mimics features of CNS GCTs. LCH frequently involve spine and is the most common cause of vertebra plana in children. A 15-year-old boy presented with progressing symptoms of polydipsia, polyuria, general headache, nausea and severe back pain. Brain MRI showed brain tumor with simultaneous involvement of suprasellar region and pineal gland. An excisional biopsy of suprasellar mass was done. The pathologic assessment confirmed the diagnosis of germinoma. Patient's treatment continued accordingly. A spine MRI, done due to persistent backache, showed a vertebra plana. We reevaluated the primary diagnosis suspecting LCH. Germinoma of CNS was confirmed and a biopsy of vertebral lesion resulted in hemangioma. Thus we report a case of CNS germinoma with co-occurrence of vertebra plana. We emphasized the importance of histopathologic diagnosis of pineal/suprasellar masses and primary investigation of other CNS regions including spine for possible metastasis or comorbidities.

Eosinophilic granuloma in the anterior mandible mimicking radicular cyst

  • Lee, Byung-Do;Lee, Wan;Lee, Jun;Son, Hyun-Jin
    • Imaging Science in Dentistry
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    • 제43권2호
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    • pp.117-122
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    • 2013
  • Eosinophilic granuloma is a common expression of Langerhans cell histiocytosis and corresponds with typical bone lesions. The radiographic appearance of eosinophilic granuloma in the jaw is variable and not specific. It may resemble periodontitis, radicular cyst, or malignancies. The purpose of this report is to describe the characteristic radiographic features of eosinophilic granuloma of a 39-year-old male. The lesion in the anterior mandible was first diagnosed as radicular cyst because the radiographic findings were ovoid radiolucent lesion with well-defined border. However, careful interpretation revealed a non-corticated border and floating tooth appearance that were the characteristic radiographic features for the differential diagnosis. Early clinical signs of eosinophilic granuloma can occur in the jaw and a bony destructive lesion might be mistaken for periodontitis or an odontogenic cystic lesion; therefore, careful interpretation of radiographs should be emphasized.

Erdheim-Chester Disease Presenting as an Anterior Mediastinal Tumor without Skeletal Involvement

  • Lee, Kanghoon;Kim, Hyeong Ryul;Roh, Jin;Ok, You Jung;Jeon, Bo Bae;Kim, Young Woong
    • Journal of Chest Surgery
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    • 제51권3호
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    • pp.223-226
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    • 2018
  • Erdheim-Chester disease (ECD) is a form of non-Langerhans cell histiocytosis that most commonly involves the skeletal system. We report an unusual case of ECD presenting as an anterior mediastinal tumor without skeletal involvement. A 60-year-old man with no remarkable medical history was referred for evaluation of a mediastinal mass. The patient underwent surgical excision of the tumor via video-assisted thoracoscopic surgery. Histologic examination revealed marked proliferation of atypical histiocytes with sclerosis, and the results of immunohistochemical staining were suggestive of ECD.

양측성, 재발성 자연기흉을 동반한 폐 조직구증 1예 (A Case of Pulmonary Histiocytosis-X Associated with Bilateral, Recurrent, and Spontaneous Pneumothorax)

  • 홍사준;안강현;이원연;공수정;용석중;신계철
    • Tuberculosis and Respiratory Diseases
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    • 제41권2호
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    • pp.152-157
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    • 1994
  • 본 저자들은 양측성으로 반복되는 자연기흉 소견을 보인 21세 남자 환자에서BAL은 시행하여 조직구내 Birbeck granule을 찾음으로써 확진할 수 있었던 골 침범을 동반한 폐 조직구증 1예를 문헌 고찰과 함께 보고하는 바이다.

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가임기 간접흡연 여성에서 발생한 폐 호산구성 육아종 1예 (A Case of Pulmonary Eosinophilic Granuloma in a Passive-Smoking Reproductive Female)

  • 신재호;김지영;오수환;박홍수;정상수;나상규;김형중;안철민;김해균;김상진;조상호
    • Tuberculosis and Respiratory Diseases
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    • 제45권2호
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    • pp.421-428
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    • 1998
  • 기흉과 미만성 간질성 폐질환의 소견을 보인 흡연력이 없는 젊은 여자 환자에서 개흉 폐생검후 면역조직화학염색을 통하여 폐 호산구성 육아종을 진단한 1예를 경험하였기에 보고하는 바이다.

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하악과두에 발생한 호산구육아종 (Eosinophilic granuloma of the mandibular condyle)

  • 최문경;허경회;이원진;오성욱;이삼선
    • Imaging Science in Dentistry
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    • 제38권1호
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    • pp.63-67
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    • 2008
  • The present study reports a case of eosinophilic granuloma of the mandibular condyle. Eosinophilic granulomas on the mandibular condyle are very rare, but there are several common clinical and radiographic presentations. The clinical presentations involve swelling on preauricular area, limitation of opening, TMJ pain, etc. The radiographic presentations involve radiolucent lytic condylar lesion with or without pathologic fracture. Sometimes new bone formations are observed. The purpose of the article is to add new cases to the literatures.

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후두에 발생한 파종성 황색종 1예 (A Case of Xanthoma Disseminatum with Laryngeal Involvement)

  • 박진수;이용우;이상혁;진성민
    • 대한후두음성언어의학회지
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    • 제26권1호
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    • pp.58-62
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    • 2015
  • Xanthoma disseminatum (XD) is a benign, non-Langerhans cell histiocytosis characterized by disseminated xanthomatous lesions with face, flexures, and mucosa. Most of XD develops in mucocutaneous lesions including skin, oral cavity and pharynx, however laryngeal involvement is uncommon. While the natural course of XD is usually benign and often self-limiting, but XD develop in critical anatomical locations may result in morbidity and mortality. Localized mucous lesions in oropharynx and larynx lead to dysphagia, dyspnea and air way obstruction. The diagnosis of XD was based on clinical, histological and immunohistochemical findings. The treatment is complex and non-consensual. Local treatment with cryotherapy, radiotherapy, surgery, and carbon dioxide lasers have been attempted with various results. Systemic medication with peroxisome proliferator-activated gamma receptors, statins, fenofibrate, chlorodeoxyadenosine, cyclophosphamide, doxycycline, and cyclosporine have also been reported, but none have proven particularly successful. A 59-year-old man presented with respiratory symptoms because of laryngeal involvement of XD. We had to remove the obstructive lesion for relieving the symptoms. We experienced XD in Larynx that was rare in otorhinolaryngology. So we report this case with review of literatures.

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