MULTIPLE MYELOMA AND OTHER PLASMA CELL DISEASES

multiplemyelomawos

POEMS AND OTHER RARE PLASMA CELL DYSCRAISAS

Selin Merih Urlu1 Sinem Civriz Bozdağ2

1Medicana International Ankara Hospital, Department of Hematology, Ankara, Türkiye
2Koç University Hospital, Department of Hematology, İstanbul, Türkiye

Merih Urlu S, Civriz Bozdağ S. POEMS and Other Rare Plasma Cell Dyscraisas. In: Sevindik ÖG, editor. Multiple Myeloma and Other Plasma Cell Dyscrasias. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.113-121.

ABSTRACT

POEMS syndrome, is a rare paraneoplastic syndrome of undefined pathogenesis, often overlooked in diagnosis because its symptoms are complex and affect multiple systems. The diagnosis is established through a combination of clinical and laboratory characteristics, and it may be overlooked if not adequately assessed. The categories in the POEMS acronym do not comprehensively represent the condition; they only contain a few features. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell dysfunction, sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. The exact incidence is unknown. The goal of treatment is to eliminate the underlying plasma cells. Because of the disease’s rarity, there aren’t many randomized clinical studies to guide treatment selection; instead, case series and case reports are used for guidance on treatment decisions. Vascular endothelial growth factor, the cytokine most closely associated with disease activity in POEMS syndrome, which is helpful for the diagnosis and evaluation of treatment response.

Keywords: POEMS; Polyneuropathy; Vascular endothelial growth factor; Castleman disease

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