• Journal of Korean Neurosurgical Society
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• 제30권sup1호
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• pp.124-127
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• 2001

Langerhans' cell histiocytosis is a rare disease showing proliferation of histiocytes in multiple organ system. Bone lesions are the most common radiolologic manifestation of Langerhans' cell hysticytosis. Extraskeletal sites of Langerhans' cell hystiocytosis involvement include the skin, lymph nodes, thymus, lungs, central nervous system, liver, pancrease, spleen, and bowel. The authors have experienced a case of Langerhans' cell histiocytosis which involved the skull. A 3-year-old female presented with soft tissue mass on right periorbital area. Plain skull X-ray showed punched out bone lesion. Computed tomography showed non-enhancing osteolytic lesion on right frontal skull. Histologic findings of an excised mass revealed pathologic features of Langerhans' cell histiocytosis.

• Clinical and Experimental Pediatrics
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• 제50권6호
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• pp.524-530
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• 2007

The recent advances in the basic hematology and immunology have significantly enhanced the understanding of histiocytic disorders. The Histiocyte Society which was established in 1985 enabled the randomized trials for these diseases, and important knowledge regarding pathogenesis, clinical presentation, diagnosis, therapy and late consequences has been obtained. The treatment of Langerhans cell histiocytosis (LCH) has varied greatly over last decades, and is still controversial. Therapy can be reduced for low risk patients, and it is possible to discriminate early the non-responding patients with risk disease who might require more intensified treatment. Current therapy of LCH recommended by the Histiocyte Society (LCH-III protocol) is activated in 2001. Hemophaocytic histiocytosis (HLH) is fatal if diagnosis is delayed and appropriate therapy is not instituted rapidly. The diagnostic criteria for HLH is revised by the Histiocyte Society for the current treatment protocol (HLH-2004) which consists of dexamethasone, etoposide, and cyclosporin in combination with intathecal methotrexate. Hematopoietic stem cell transplantation is usually necessary for the primary HLH and recurrent secondary HLH.

• Journal of Korean Neurosurgical Society
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• 제43권6호
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• pp.304-306
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• 2008

Langerhans' cell histiocytosis (LCH) is a rare immunologic disorder characterized by histiocyte proliferation in multiple organ systems. Eosinophilic granuloma, a benign bone lesion, represents a focal form of LCH. We experienced a case of Langerhans' cell histiocytosis in a patient who presented with intracranial epidural hematoma and cyst on the midline of the frontal skull. A 10-year-old boy presented with a rapidly growing large scalp mass on the midline frontal area after mild head trauma. The scalp mass was painless and immobile. Plain skull x-ray showed a punched-out bone lesion. Computed tomography and magnetic resonance imaging showed a non-enhancing osteolytic lesion presenting with an epidural hematoma and cyst on the midline of the frontal skull. The lesion of the skull was completely resected and the patient's recovery was uneventful. The acute presentation of a solitary eosinophilic granuloma of skull with an epidural hematoma has been described in only five cases in the literature and we report the first case of LCH presenting as an intracranial epidural hematoma on frontal area.

• 대한골관절종양학회지
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• 제19권1호
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• pp.28-32
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• 2013

Erdheim chester disease (ECD)은 골격계 및 다발성 장기에 발생하는 매우 드문 비 랑거한스세포 조직구증(Langerhans cell histiocytosis,LCH)으로 구분되는 질환으로 진행성이며 때론 치명적인 결과를 야기한다. 하지만 이는 LCH 혹은 다발성 골 전이로 오인되기 쉬우며 진단 또한 극히 어렵다. 국내에서는 1999년에 10예가 처음으로 보고되었을 뿐이며 특히 정형외과적으로 접근 및 보고는 문헌상 영어권에서 극히 소수의 문헌만이 존재하며 국내에는 보고된 예가 없다. 저자들은 슬관절 및 하지통은 주소로 본과에 협진된 환자에 대해 골 조직검사 시행하였으며 이에 대한 진단적 경험을 문헌 고찰과 함께 보고하고자 한다.

• 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.

• 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.

• 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.

• 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.

• Tuberculosis and Respiratory Diseases
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• 제67권4호
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• pp.345-350
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• 2009

Erdheim-Chester disease (ECD) is a proliferative non-Langerhans cell histiocytosis of multiple organs. This is a rare disease of unknown etiology with a high mortality. We present the case report of a 26-year-old man diagnosed with ECD. He was referred to our hospital with elevated levels of aminotransferases. Although the diagnosis was uncertain, the patient was lost to follow up at that time. One year later, the patient returned to the hospital with generalized edema. Although a specific bone lesion was not found, the patient was experiencing the following: glomerulonephritis, aplastic anemia, hepatitis, and lung involvement. A lung biopsy was performed: the immunohistochemical stain were positive for CD68 and negative for S-100 protein and CD1a. We diagnosed as the patient as havinf ECD. Approximately 50% of ECD cases present with extraskeletal involvement. ECD should be considered as part of the differential diagnosis when multiple organs are involved.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제2권2호
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• pp.204-210
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• 1999

목 적: 소아암 환자들을 대상으로 항암치료 전후의 영양상태를 평가하고, 항암 치료기간 중 발생하는 식욕부진시에 섭취량 및 기호 식품을 조사함으로써 향후 이들 환자의 보다 나은 영양관리를 위한 지침을 마련하고자 본 연구를 시행하였다. 방 법: 1996년 1월부터 1998년 12월까지 동아대학교병원 소아과에서 백혈병 및 소아암으로 진단받고 항암 치료를 시작하는 환자 20명을 대상으로 하였다. 이들 환자의 항암 치료 전과 21일 후의 영양상태 평가를 위하여 인체 계측과 생화학적 검사(총 단백질, 알부민, AST, ALT, BUN, creatinine)를 시행하였다. 항암 치료 기간중 식욕저하 기간과 원인 및 평소에 선호하는 식품과 치료 후에 선호하는 식품에 관한 기호도를 설문조사하였다. 결 과: 1) 대상 환자는 남아 10명, 여아 10명이었으며, 연령은 3세에서 15세로 정중 연령이 6세이었다. 질환별로는 급성 림프구성 백혈병 11명, 비호지킨 림프종 2명, 조직구증식증 2명, 읠름씨 종양 2명, 뇌종양 2명, 횡문근 육종 1명이었다. 2) 항암 치료를 받는 동안 체중 감소가 있었던 경우가 8명 (40%), 체중 증가가 있었던 경우가 5명(25%), 체중의 변화가 없었던 경우가 7명 (35%)이었다. 3) 항암 치료를 받는 동안 실제 식사 섭취량은 1일 평균 450 Kcal (310~600 Kcal)이었다. 4) 항암 치료 시작 당시 혈청 알부민은 3.1~4.4 g/dL의 분포로 정상보다 낮은 범위에 있었으며 항암 치료 21일째는 2.7~5.0 g/dL로서 통계적 차이는 보이지 않았다. 혈청 ALT는 항암 치료전보다 의미있게 증가하였으나(p<0.05), 혈청 AST, BUN, creatinine, 총 단백질은 의미있는 변화가 없었다. 5) 항암 치료기간 동안 식욕저하의 원인으로서는 구토가 37%로 가장 많았으며 메스꺼움, 씹기 곤란함, 연하 곤란, 음식 혐오 순이었다. 6) 식욕 저하를 호소할 때 먹은 식품으로서 흰죽, 전복죽 등의 죽 종류를 가장 많이 먹었고, 곰국, 장어 등의 보양식을 그 다음으로 섭취하였으며 인스턴트 식품도 섭취하였다. 7) 항암 치료기간 동안 탄산 음료를 가장 좋아하였고, 맵고 자극적인 식품을 좋아하는 것으로 나타났으며, 싫어하는 식품으로는 평소 싫어하는 식품과 마찬가지로 야채류로 나타났다. 결 론: 항암 화학요법을 받고 있는 소아들은 소모성 질환으로 인해 영양상태가 더욱 나빠지기 쉬우므로 항암 치료를 받는 기간동안 보다 적극적이고 체계적인 환자의 식사 섭취량 조사 및 환자 상태와 기호에 적합한 형태의 식사종류 개발이 필요하며, 환자나 보호자들에게도 적절한 식사 종류 및 식사요령을 교육, 홍보하여 환자의 식사 섭취량 증가를 통한 영양 상태 개선이 이루어지도록 해야하겠다.