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KMID : 1100720190390060561
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Annals of Laboratory Medicine
2019 Volume.39 No. 6 p.561 ~ p.565
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POEMS Syndrome: Bone Marrow, Laboratory, and Clinical Findings in 24 Korean Patients
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Shim Hyo-Eun
Seol Chang-Ahn
Park Chan-Jeoung
Cho Young-Uk
Seo Eul-Ju
Lee Jung-Hee
Yoon Dok-Hyun
Suh Cheol-Won
Park Sang-Hyuk
Jang Seong-Soo
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Abstract
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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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KEYWORD
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POEMS syndrome, Clinical manifestations, Plasma cell, Flow cytometry, Bone marrow
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