Ann Lab Med.  2019 Nov;39(6):561-565. 10.3343/alm.2019.39.6.561.

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

• Shim H
• Seol CA
• Park CJ
• Cho YU
• Seo EJ
• Lee JH
• Yoon DH
• Suh CW
• Park SH
• Jang S

• KMID: 2450951
• DOI: http://doi.org/10.3343/alm.2019.39.6.561

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.

Keyword

POEMS syndrome; Clinical manifestations; Plasma cell; Flow cytometry; Bone marrow

MeSH Terms

Bone Marrow*
Diagnosis
Flow Cytometry
Humans
Hyperplasia
Medical Records
Megakaryocytes
Paraneoplastic Syndromes
Paraproteinemias
Plasma Cells
POEMS Syndrome*
Polyneuropathies
Skin

Figure

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  Fig. 1 Bone marrow finding of PCs rimming around a lymphocyte aggregate. (A) The lymphocytic aggregates show a germinal center (H&E stain, ×400). PCs form a rim around the lymphocytic collection detected by immunohistochemistry of (B) CD138, (C) κ, and (D) λ.Abbreviations: H&E, hematoxylin and eosin; PC, plasma cell.

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