폐와 척추를 침범한 랑게르한스 세포 조직구증 1예
A Case of Langerhans Cell Histiocytosis Involving the Lung and Spine of an Adult Patient
1
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
랑게르한스 세포 조직구증은 드문 조직구 질환의 하나로서 랑게르한스 세포가 증식하여 뼈를 비롯한 여러 장기를 침범하는 질환이다. 성인에서 폐와 함께 척추를 침범한 경우는 드물게 보고되고 있다. 저자들은 흡연력이 있는 젊은 남성에서 폐 병변은 금연에 의해 호전되었지만 척추 병변은 금연에도 불구하고 악화되어 심한 통증을 유발하고 척추 안정성에 문제를 보여 이를 수술적으로 치료한 1예를 경험하였기에 보고하는 바이다.
Abstract
Langerhans cell histiocytosis (LCH) is a proliferative disease that shares phenotypic characteristics with Langerhans cells (LCs) and involves multiple organs. Here, we report a case of LCH in a 36-year-old male simultaneously involving the lungs and thoracic spine. Chest computed tomography (CT) showed multifocal thin-walled cysts and ill-defined nodular opacities primarily in the bilateral upper and middle lung lobes that fluctuated with smoking. Despite improvement of pulmonary lesions after smoking cessation, osteolytic lesion of thoracic vertebra was aggravated. Symptoms were not improved after nerve block, suggesting vertebral instability that necessitated surgical mass removal and posterior fusion. Microscopically diffuse proliferation of immature, rounded LCs and inflammatory cell infiltration was noted. Although pulmonary LCH in an adult is a benign disease that is usually resolved spontaneously after smoking cessation, surgical treatment should be considered in a case of spinal LCH.
참고문헌 (7)
- Vassallo R, "Clinical outcomes of pulmonary Langerhans'-cell histiocytosis in adults", N Engl J Med, 346, pp.484~490 (2002)
- Grois N, "Risk factors for diabetes insipidus in langerhans cell histiocytosis", Pediatr Blood Cancer, 46, pp.228~233 (2006)
- 김철, "국내 폐 랑게르한스세포 조직구증(Pulmonary Langerhans Cell Histiocytosis) 의 임상양상", Tuberculosis and Respiratory Diseases, 66(2), pp.98~103 (2009), 대한결핵및호흡기학회
- 안세환, "Adult Langerhans Cell Histiocytosis of the Spine Presenting with Neurological Deficits: A Case Report", 대한척추신경외과학회지, 7(3), pp.199~201 (2010), 대한척추신경외과학회
- Yousem SA, "Pulmonary Langerhans' cell histiocytosis : molecular analysis of clonality", Am J Surg Pathol, 25, pp.630~636 (2001)
- Badalian-Very G, "Recent advances in the understanding of Langerhans cell histiocytosis", Br J Haematol, 156, pp.163~172 (2012)
- Tazi A, "Adult pulmonary Langerhans' cell histiocytosis", Eur Respir J, 27, pp.1272~1285 (2006)