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A Case of Pulmonary Amyloidosis Mimicking Lymphangitic Lung Carcinomatosis

림프관성 폐 암종증으로 오인된 폐 아밀로이드증 1예

  • Shin, Beomsu (Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Ko, Jaehoon (Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Lee, Seong Soo (Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Lim, Kyung-Suk (Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Han, Joung Ho (Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Chung, Man Pyo (Division of Pulmonary and Critical Care Medicine, Departments of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Jeong, Byeong-Ho (Division of Pulmonary and Critical Care Medicine, Departments of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine)
  • 신범수 (성균관대학교 의과대학 삼성서울병원 내과) ;
  • 고재훈 (성균관대학교 의과대학 삼성서울병원 내과) ;
  • 이성수 (성균관대학교 의과대학 삼성서울병원 내과) ;
  • 임경숙 (성균관대학교 의과대학 삼성서울병원 내과) ;
  • 한정호 (성균관대학교 의과대학 삼성서울병원 병리과) ;
  • 정만표 (성균관대학교 의과대학 삼성서울병원 호흡기내과) ;
  • 정병호 (성균관대학교 의과대학 삼성서울병원 호흡기내과)
  • Received : 2013.08.22
  • Accepted : 2013.09.12
  • Published : 2014.03.01

Abstract

Amyloidosis is defined by tissue deposits of amyloid, which cause both functional and structural damage to organs. Three histopathological types of pulmonary amyloidosis have been described: tracheobronchial, nodular parenchymal, and diffuse parenchymal or alveolar septal types. Diffuse alveolar septal involvement of the lung is a rare form of primary pulmonary amyloidosis, with radiological features similar to those of pulmonary edema, pulmonary fibrosis, and lymphangitic carcinomatosis. Therefore, it is always challenging to diagnose alveolar septal involvement of pulmonary amyloidosis. Here, we report a case of alveolar septal involvement of pulmonary amyloidosis mimicking lymphangitic carcinomatosis, which was diagnosed from a transbronchial lung biopsy.

아밀로이드증은 불용성의 섬유단백이 세포 바깥 부위에 침착 되어 발생하는 드문 질환으로 해당 장기의 기능적 구조적 손상을 일으킨다. 폐 아밀로이드증은 침범형태에 따라 기관기관지형, 결절실질형, 미만성실질형 또는 폐포벽형으로 나눌 수 있다. 이 중에 폐포벽형 폐 침범은 매우 드물고 방사선학적 형태가 폐부종, 폐섬유화증, 림프관성 암종증과 비슷하여 진단을 내리는 데 어려움을 준다. 저자들은 TBLB를 통하여 폐포벽형태의 폐 아밀로이드증을 진단하고 다발성골수종 동반을 진단 및 아밀로이드증의 신경계 침범을 의심할 수 있었던 증례를 경험하였기에 문헌고찰과 함께 보고한다.

Keywords

References

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Cited by

  1. Single Nodular Pulmonary Amyloidosis: Case Report vol.78, pp.4, 2014, https://doi.org/10.4046/trd.2015.78.4.385