지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 저널정보
- 대한병리학회 Journal of Pathology and Translational Medicine Journal of Pathology and Translational Medicine 제52권 제3호
- 발행연도
- 2018.1
- 수록면
- 183 - 190 (8page)
이용수
초록· 키워드
오류제보하기
Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis and multisystem disease. First described in 1930, there are no more than 750 cases reported. The etiology remains unknown, but a majority of cases of ECD and Langerhans cell histiocytosis were found to have clonal mutations involving genes of the mitogen-activated protein kinase pathway. We recently encountered a 53-year-old male patient with extensive ECD involving the systemic lymph nodes, pleura, liver, and long bones clinically mimicking malignant lymphoma. Biopsies were performed at multiple sites, including a pleural mass, an external iliac lymph node, bone marrow, and the liver. Based on histopathological and immunohistochemical findings of positivity for CD68 and negativity for CD1a and S-100, the patient was diagnosed with ECD. Interferon-α was administered as the first-line treatment, but the patient rapidly progressed to hepatic failure after 2 months of treatment. We report this rare case of ECD clinically mimicking malignant lymphoma and diagnosed by careful pathological review.
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등록된 정보가 없습니다.
참고문헌 (20)
참고문헌 신청
Campochiaro C / 2015 / Erdheim-Chester disease / Eur J Intern Med 26:223~229
임진 / 2016 / A Unique Case of Erdheim-Chester Disease with Axial Skeleton, Lymph Node, and Bone Marrow Involvement / Cancer Research and Treatment 48(1):415~421
Bindra J / 2014 / Erdheim-Chester disease: an unusual presentation of an uncommon disease / Skeletal Radiol 43:835~840
Pavlidakey PG / 2011 / Erdheim-chester disease associated with marginal zone lymphoma and monoclonal proteinemia / Case Rep Hematol 2011
Haroche J / 2007 / Erdheim-Chester disease / Presse Med 36:1663~1668
임진 / 2016 / A Unique Case of Erdheim-Chester Disease with Axial Skeleton, Lymph Node, and Bone Marrow Involvement / Cancer Research and Treatment 48(1):415~421
Bindra J / 2014 / Erdheim-Chester disease: an unusual presentation of an uncommon disease / Skeletal Radiol 43:835~840
Pavlidakey PG / 2011 / Erdheim-chester disease associated with marginal zone lymphoma and monoclonal proteinemia / Case Rep Hematol 2011
Haroche J / 2007 / Erdheim-Chester disease / Presse Med 36:1663~1668
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