POEMS Syndrome: Bone Marrow, Laboratory, and Clinical Findings in 24 Korean Patients

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 th...

Full description

Saved in:
Bibliographic Details
Published in:Annals of laboratory medicine 2019, 39(6), , pp.561-565
Main Authors: Shim, Hyoeun, 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, Seongsoo
Format: Article
Language:English
Subjects:
Adult
Aged
Aged, 80 and over
Antigens, CD19 - metabolism
Bone Marrow - metabolism
Bone Marrow - pathology
Brief Communication
Female
Glycoproteins - analysis
Humans
Immunophenotyping
Lymphocytes - cytology
Lymphocytes - metabolism
Male
Middle Aged
Paraproteinemias - diagnosis
POEMS Syndrome - diagnosis
Republic of Korea
Retrospective Studies
Young Adult
병리학
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:2234-3806
2234-3814
DOI:10.3343/alm.2019.39.6.561