Ann Lab Med 2019; 39(6): 561-565  https://doi.org/10.3343/alm.2019.39.6.561
POEMS Syndrome: Bone Marrow, Laboratory, and Clinical Findings in 24 Korean Patients
Hyoeun Shim , M.D.1*, Chang Ahn Seol , M.D.2*, Chan-Jeoung Park , M.D.2, Young-Uk Cho , M.D.2, Eul-Ju Seo , M.D.2, Jung-Hee Lee , M.D.3, Dok Hyun Yoon , M.D.3, Cheol Won Suh , M.D.3, Sang Hyuk Park , M.D.4, and Seongsoo Jang , M.D.2
1Department of Laboratory Medicine, National Cancer Center, Goyang, Korea; Departments of 2Laboratory Medicine and 3Medicine, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea; 4Department of Laboratory Medicine, University of Ulsan College of Medicine, Ulsan University Hospital, Ulsan, Korea
Corresponding author: Chan-Jeoung Park, M.D.
Department of Laboratory Medicine, University of Ulsan College of Medicine and Asan Medical Center, 88 Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea
Tel: +82-2-3010-4508 Fax: +82-2-478-0884 E-mail: cjpark@amc.seoul.kr
*These authors contributed equally to this work.
Received: December 20, 2018; Revised: April 19, 2019; Accepted: May 28, 2019; Published online: November 1, 2019.
© Korean Society for Laboratory Medicine. All rights reserved.

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
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.
Keywords: POEMS syndrome, Clinical manifestations, Plasma cell, Flow cytometry, Bone marrow



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