• Pediatric Infection and Vaccine
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• 제16권2호
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• pp.220-223
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• 2009

랑게르한스 세포 조직구증은 소아에서 매우 드믄 질환이지만 발병시 경과나 증세가 매우 다양하게 나타날 수 있으며 다른 염증성 질환으로 오인될 소지가 있다. 따라서 임상 의사들은 이 병의 특징이나 경과에 대해 잘 알고 있어 병의 진단이나 치료가 늦어지지 않도록 주의하여야 하겠다. 저자들은 최근 안와주위 부종과 열감을 주소로 내원한 1세 남아에게서 조직 검사상 랑게르한스 세포 조직구증이 진단 되었기에 문헌고찰과 함께 보고하는 바이다. 항생제 치료 수일 후에도 호전이 없는 안와 염증의 경우 랑게르한스 세포 조직구증을 반드시 감별 질환의 하나로 고려해야 한다.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제31권3호
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• pp.249-253
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• 2009

Langerhans cell histiocytosis(LCH), previously known as histiocytosis X, is rare, proliferative disorder in which the accumulation of pathologic Langerhans cell leads to tissue infiltration and destruction. The jaw is involved 10~20% of all LCH and most common oral symptoms are jaw swelling or a palpation mass. Most of subjects are males and disease appears to start before the age of 10 years. An 11 years old girl was referred from pediatric department. Histological examination confirmed the diagnosis LCH. She was treated intra-lesional injection of corticosteroid. The overall outcome was excellent. After a follow-up period of 20 month, the patient present with no evidence of residual disease on Panoramic view and whole body bone scan. We report this early recognized LCH case treated by corticoid injection in good progress with literature review.

• 대한두개안면성형외과학회지
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• 제10권1호
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• pp.37-39
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• 2009

Purpose: Langerhans cell histiocytosis is a heterogenous group of Langerhans cell proliferative disorders and includes eosinophilic granuloma, Letterer-Siwe diseases, and Hand-Schuller Christian disease. We report a case of eosinophilic granuloma on frontal area. Methods: A 17-year-old male presented with swelling and tenderness on Lt. frontal and periorbital area. CT and MRI showed a $33{\times}25mm$ sized mass that involved Lt. frontal calvarium, frontotemporal meninges, and orbital roof. Results: Total excision of the mass and adjacent soft tissue, calvarium, and orbital roof was performed. Orbital roof defect was reconstructed with absorbable plate and calvarial defect was done with outer cortex of temporal bone flap. The histology revealed proliferation of histiocytes and eosinophils. Immunologically, these histiocytic cells expressed S-100 protein and CD1a. The patient is currently taking conservative treatment. Conclusion: The severity of these disease and their prognosis and treatments are various. For unifocal cranial Langerhans cell histiocytosis, complete excision is the treatment of choice. We report this case with review of literature.

• Journal of Chest Surgery
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• 제34권6호
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• pp.506-510
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• 2001

Langerhans 세포 조직구증(LCH, Langerhans\` Cell Histiocytosis)은 Langerhans\` cell histiocyte의 이상 증식을 특징으로 하는 원인 불명의 질환이다. 이 질환은 eosinophilic granuloma, Hand-Sch ller-Christian씨 병, Letterer-Siwe병을 포함하는 것으로 과거에는 histiocytosis X로 불리던 질환이다. 피부, 림프절, 골, 골수 및 체내 모든 조직과 기관을 침범할 수 있으나 국내에서 흉벽에서 발생된 예는 보고된 증례가 많지않다. 18개월 된 남자 환아에서 흉벽의 늑골에서 기원하여 골용해 소견을 동반한 종괴가 있어 수술적 절제한 후 LCH로 확진된 증례가 있어 문헌고찰과 함께 보고하는 바이다

• Maxillofacial Plastic and Reconstructive Surgery
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• 제19권1호
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• pp.55-60
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• 1997

Langerhans Cell Histiocytosis(Idiopathic Histiocytosis, Histiocytosis-X) is most often found in children and young adults, and cell proliferation with specific phenotype shows ultrastructural and immunohistochemical similarities with Langerhans Cells that normally exist in epithelium and mucosa. This disease occurs as single or multiple lesions in skull, ribs, vertebrae, mandible and long bones, and when it involves mandible, clinical sign and symptoms such as bone swelling and pain are noticed. When it involves alveolar bone, severe tooth mobility as well as gigival inflammation, proliferation, and ulceration are commonly found, and so it is not easy to differentiate it from general inflammatory diseases. Any local lesion at the tooth apex on the x-ray view needs to be differentiated from inflammatory disease, and multiple lesions from multiple ostoeoma and chronic multifocal osteomyelitis. This case is LCH in 51-year-old male patient ; this is a rare case, for the patient belongs to an age group with very low incidence rate of the disease. although three-timed biopsy tests and longterm observation at two university hospitals, it was misdiagnosed as multifocal osteomyelitis.

• 대한세포병리학회지
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• 제2권2호
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• pp.153-159
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• 1991

저자들은 세포학적 검사로 진단이 가능하였던 2예의 Langerhans 세포 조직구증의 세포학적 소견을 기술하였다. 한 예는 3개월된 남아의 구진성 피부병소로 부터 얻은 찰과 도말 표본에서, 다른 예는 3세된 남아의 종창된 경부림프절의 세침천자 세포학적 표본에서 각각 특징적인 조직구의 도말배경을 관찰함으로써 진단이 가능하였다. 진단적인 세포학적 표본의 검색에서 도말된 세포성분은 풍부하고, 도말배경은 깨끗하였다. 특징적인 조직구는 길죽한 핵의 한쪽이 약간 함몰되고, 긴 구열을 가지며, 핵막은 얇고, 염색질은 섬세하며, 핵소체는 인정되지 않았다. 호산성의 세포질은 풍부하고, 활동적인 탐식작용의 증거는 관찰되지 아니하였다. 특징적인 조직구와 더불어 림프구와 드물게 호산구가 도말배경을 이루고 있었다.

• 대한골관절종양학회지
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• 제19권2호
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• pp.78-82
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• 2013

랑게르한스 세포 조직구증(Langerhans' cell histiocytosis)은 단핵구계에 속하는 정상 조직구들이 과도하게 증식하여 다양한 임상 경과와 치료결과를 보이는 질환 군으로 알려져 있다. 특히, 척추에 침범하는 경우에는 단발 혹은 다발성의 골 용해를 특징으로 한다. 병변의 침범 부위, 진행 정도 및 동반 증상에 따라 단순 경과 관찰에서부터 방사선 치료, 화학요법 및 수술적 치료에 이르기까지 다양한 치료법들이 적용되어 왔으나, 아직까지 확립된 치료법은 없는 것으로 알려져 있다. 저자들은 경추와 요추를 동시에 침범하면서 척추 내 골수 파괴 소견과 병적 골절을 동반한 단일조직 랑게르한스 세포 조직구증 환자에 대해 수술적 치료 및 전신화학요법을 시행한 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Journal of Yeungnam Medical Science
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• 제22권2호
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• pp.259-265
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• 2005

Central diabetes insipidus (DI) is a syndrome characterized by thirst, polydipsia and polyuria. Langerhans cell histiocytosis is one of the etiologies of DI. Recently we experienced a central DI associated with Langerhans cell histiocytosis. The 44 years old female patient complained right hip pain, polydipsia and polyuria. We carried out water deprivation test. After vasopressin injection, urine osmotic pressure was increased from 109 mOsmol/kg to 327 mOsmol/kg (300%). Brain MRI showed a thickened pituitary stalk and air bubble like lesions sized with 5cm, 7cm was shown on fifth L-spine and right hip bone at hip bone CT. CT guided biopsy revealed abnormal histiocytes proliferation and abundant lymphocytes. The final diagnosis was central DI associated with systemic Langerhans cell histiocytosis invading hip bone, L-spine and pituitary stalk. Desmopressin and etoposide chemotherapy were performed to the patient.

• Investigative Magnetic Resonance Imaging
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• 제24권1호
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• pp.61-65
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• 2020

Langerhans cell histiocytosis (LCH) is generally considered a childhood disease that exhibits various nonspecific clinical and radiological manifestations that mimic infection or malignancy. Here, we present a case of LCH involving the rib in an adult patient. CT and MRI revealed an expansile lytic lesion with periosteal reaction on the left 8th rib, suggesting a malignant bone tumor. Surgical resection was performed and histopathological examination was consistent with LCH. Owing to its rare occurrence in adults and nonspecific aggressive features, LCH should be included in the differential diagnosis of an aggressive-appearing rib lesion in both adults and children.

• Journal of Korean Neurosurgical Society
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• 제47권6호
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• pp.458-460
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• 2010

Langerhans cell histiocytosis (LCH), a disorder of the phagocytic system, is a rare condition. Moreover, spinal involvement causing myelopathy is even rare and unusual. Here, we report a case of atypical LCH causing myelopathy, which was subsequently treated by corpectemy and fusion. A 5-year-old boy presented with 3 weeks of severe neck pain and limited neck movement accompanying right arm motor weakness. CT scans revealed destruction of C7 body and magnetic resonance imaging showed a tumoral process at C7 with cord compression. Interbody fusion using cervical mesh packed by autologus iliac bone was performed. Pathological examination confirmed the diagnosis of LCH. After the surgery, the boy recovered from radiating pain and motor weakness of right arm. Despite the rarity of the LCH in the cervical spine, it is necessary to maintain our awareness of this condition. When neurologic deficits are present, operative treatment should be considered.