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

Affiliations
  • 1Department of Laboratory Medicine, National Cancer Center, Goyang, Korea.
  • 2Department of Laboratory Medicine, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea. cjpark@amc.seoul.kr
  • 3Department of Medicine, 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.

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

  • 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.


Reference

1. Dispenzieri A. POEMS syndrome. Blood Rev. 2007; 21:285–299. PMID: 17850941.
2. Swerdlow SH, Campo E, et al. WHO classification of tumors of haematopoietic and lymphoid tissues. Lyon: IARC Press;2017.
3. Soubrier M, Dubost JJ, Serre AF, Ristori JM, Sauvezie B, Cathebras P, et al. Growth factors in POEMS syndrome: evidence for a marked increase in circulating vascular endothelial growth factor. Arthritis Rheum. 1997; 40:786–787. PMID: 9125266.
4. Chacko JM, Spinner WD. Paraproteinemic neuropathies. J Clin Neuromuscul Dis. 2006; 7:185–197. PMID: 19078807.
5. Lozeron P, Adams D. Monoclonal gammopathy and neuropathy. Curr Opin Neurol. 2007; 20:536–541. PMID: 17885441.
6. Dispenzieri A. POEMS syndrome: update on diagnosis, risk-stratification, and management. Am J Hematol. 2012; 87:804–814. PMID: 22806697.
7. Dao LN, Hanson CA, Dispenzieri A, Morice WG, Kurtin PJ, Hoyer JD. Bone marrow histopathology in POEMS syndrome: a distinctive combination of plasma cell, lymphoid, and myeloid findings in 87 patients. Blood. 2011; 117:6438–6444. PMID: 21385854.
8. Dispenzieri A. POEMS syndrome: 2014 update on diagnosis, risk-stratification, and management. Am J Hematol. 2014; 89:214–223. PMID: 24532337.
9. Li J, Zhou DB, Huang Z, Jiao L, Duan MH, Zhang W, et al. Clinical characteristics and long-term outcome of patients with POEMS syndrome in China. Ann Hematol. 2011; 90:819–826. PMID: 21221584.
10. Gherardi RK, Bélec L, Fromont G, Divine M, Malapert D, Gaulard P, et al. Elevated levels of interleukin-1 beta (IL-1 beta) and IL-6 in serum and increased production of IL-1 beta mRNA in lymph nodes of patients with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome. Blood. 1994; 83:2587–2593. PMID: 8167341.
11. Feinberg L, Temple D, de Marchena E, Patarca R, Mitrani A. Soluble immune mediators in POEMS syndrome with pulmonary hypertension: case report and review of the literature. Crit Rev Oncog. 1999; 10:293–302. PMID: 10654928.
12. Koga H, Tokunaga Y, Hisamoto T, Sujaku K, Mitsuyama K, Matsuo K, et al. Ratio of serum vascular endothelial growth factor to platelet count correlates with disease activity in a patient with POEMS syndrome. Eur J Intern Med. 2002; 13:70–74. PMID: 11836087.
13. Pitchford SC, Lodie T, Rankin SM. VEGFR1 stimulates a CXCR4-dependent translocation of megakaryocytes to the vascular niche, enhancing platelet production in mice. Blood. 2012; 120:2787–2795. PMID: 22653973.
14. Nakanishi T, Sobue I, Toyokura Y, Nishitani H, Kuroiwa Y, Satoyoshi E, et al. The Crow-Fukase syndrome: a study of 102 cases in Japan. Neurology. 1984; 34:712–720. PMID: 6539431.
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