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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
- 발행연도
- 2019.1
- 수록면
- 561 - 565 (5page)
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초록· 키워드
오류제보하기
POEMS syndrome is a rare paraneoplastic syndrome, which includes polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes due to plasma cell (PC) neoplasm. Diagnosis of this disease is challenging because of its rarity and complex clinical manifestations. We attempted to identify the key clinical features and characteristic bone marrow (BM) findings of POEMS syndrome, by reviewing the medical records and BM analyses of 24 Korean patients. Frequent clinical manifestations included polyneuropathy (100%), monoclonal gammopathy (100%), organomegaly (92%), extravascular volume overload (79%), and endocrinopathy (63%). The BM analyses revealed mild PC hyperplasia (median PCs: 5.5%) and frequent megakaryocytic hyperplasia (88%), megakaryocyte clusters (88%), and hyperlobation (100%). Flow cytometry of BM aspirates using CD138/CD38/CD45/CD19/CD56 showed normal (67%, 4/6) or neoplastic PC immunophenotypes (33%, 2/6). A diagnosis of POEMS syndrome must be considered when a patient suspected of having PC dyscrasia shows the above clinical presentation and BM findings.
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참고문헌 (14)
참고문헌 신청
Dispenzieri A / 2007 / POEMS syndrome / Blood Rev 21:285~299
Swerdlow SH / 2017 / WHO classification of tumors of haematopoietic and lymphoid tissues / IARC Press
Soubrier M / 1997 / Growth factors in POEMS syndrome : evidence for a marked increase in circulating vascular endothelial growth factor / Arthritis Rheum 40:786~787
Chacko JM / 2006 / Paraproteinemic neuropathies / J Clin Neuromuscul Dis 7:185~197
Lozeron P / 2007 / Monoclonal gammopathy and neuropathy / Curr Opin Neurol 20:536~541
Swerdlow SH / 2017 / WHO classification of tumors of haematopoietic and lymphoid tissues / IARC Press
Soubrier M / 1997 / Growth factors in POEMS syndrome : evidence for a marked increase in circulating vascular endothelial growth factor / Arthritis Rheum 40:786~787
Chacko JM / 2006 / Paraproteinemic neuropathies / J Clin Neuromuscul Dis 7:185~197
Lozeron P / 2007 / Monoclonal gammopathy and neuropathy / Curr Opin Neurol 20:536~541
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