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Single System Langerhans' Cell Histiocytosis with Multifocal Bone Lesions and Pathologic Fracture: A Case Report

다발성 골 침습과 병적 골절을 동반한 단일조직 랑게르한스 세포 조직구증: 증례 보고

  • Hur, Jae-Seung (Department of Orthopaedic Surgery, Guri Hospital, Hanyang University College of Medicine) ;
  • Kim, Hong-Sik (Department of Orthopaedic Surgery, Guri Hospital, Hanyang University College of Medicine) ;
  • Park, Yong-Wook (Department of Pathology, Guri Hospital, Hanyang University College of Medicine) ;
  • Pyo, Ju-Yeon (Department of Pathology, Guri Hospital, Hanyang University College of Medicine) ;
  • Lee, Young-Ho (Department of Pediatrics, Hanyang University College of Medicine) ;
  • Park, Ye-Soo (Department of Orthopaedic Surgery, Guri Hospital, Hanyang University College of Medicine)
  • 허재승 (한양대학교 의과대학 구리병원 정형외과학교실) ;
  • 김홍식 (한양대학교 의과대학 구리병원 정형외과학교실) ;
  • 박용욱 (한양대학교 의과대학 구리병원 병리학교실) ;
  • 표주연 (한양대학교 의과대학 구리병원 병리학교실) ;
  • 이영호 (한양대학교 의과대학 소아청소년과학교실) ;
  • 박예수 (한양대학교 의과대학 구리병원 정형외과학교실)
  • Received : 2013.10.21
  • Accepted : 2013.11.29
  • Published : 2013.12.31

Abstract

Langerhans cell histiocytosis is known as one of the diseases related to excessive proliferation of normal monocytes and has the variety of clinical courses and treatment. Especially, in cases with the spine, it shows a feature of single or multiple osteolysis. According to the location, disease progression and concomitant symptom, variety of treatments (observation, radiotherapy, chemotherapy, surgery, etc.) have been attempted, however, appropriate treatment has not been established yet. The authors introduce the case of single system Langerhans cell histiocytosis which involves cervical and lumbar vertebrae simultaneously with bone marrow destruction and pathologic fracture.

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.

Keywords

References

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