Diagnostic Labyrinth of POEMS Syndrome: An Analysis of Real-World Clinical Practice

A.A. Tsynchenko, Olga Vladislavovna Pirogova, Iu.S. Osipov, O.V. Kudyasheva, Yu.R. Zalyalov, E.I. Lopatina, M.V. Chernous, N.V. Stepanova, T.V. Krinitsina, A.Yu. Polushin, Ya.B. Skiba, V.V. Kosmin, I.S. Moiseev, G.N. Salogub, A.D. Kulagin,

DOI:

https://doi.org/10.21320/2500-2139-2026-19-3-292-301

BACKGROUND. РOEMS syndrome is a rare multisystem syndrome associated with monoclonal proliferation of plasma cells or B-lymphocytes and based on cytokine-mediated pathogenetic mechanism. Despite the clear diagnostic criteria, timely verification of the diagnosis is challenging due to the extreme variability of clinical features.

AIM. To study clinical and laboratory profile as well as diagnostic routes of patients with РOEMS syndrome.

MATERIALS & METHODS. This retrospective observational study enrolled 40 patients with newly diagnosed РOEMS syndrome treated at two major centers for hematology in the period from 01.2016 to 09.2025.

RESULTS. On the date of POEMS syndrome diagnosis, the median age of patients was 58 years (range 28–80 years), and median time from symptom manifestation to diagnosis was 23 months (range 1–146 months). On initial presentation at the Federal center, 30 % of patients were severely disabled and completely immobilized. All of them met two essential criteria: polyneuropathy and monoclonal gammopathy. In the vast majority of cases, polyneuropathy manifested itself in a sensory and motor form (87.5 %) with predominantly mixed axonal and demyelinating lesions (79.4 %). Paraprotein was identified in 97.5 % of patients, however in 30 % of them the pathologic clone of plasma cells was not detected by standard techniques. Other manifestations of POEMS syndrome include bone abnormalities (62.5 %), increased VEGF level (73 %), organomegaly (75 %), endocrinopathy (60 %), skin lesions (52.5 %), extravascular fluid overload (47.5 %), and thrombocytosis/polycythemia (45 %). Prior to diagnosis verification, on average, patients were examined by 3 (range 1–6) medical specialists, most often by a neurologist (62.5 %). At the diagnostic stage, chronic inflammatory demyelinating polyneuropathy (32.5 %) was widely and wrongly assumed. After examination by related specialists, 80 % of patients were referred to a hematologist. In 50 % of cases, lymphoproliferative diseases were identified, however, without verification of POEMS syndrome. In 20 % of patients, myeloproliferative neoplasia was wrongly diagnosed.

CONCLUSION. For timely diagnosis of POEMS syndrome, medical specialists should raise awareness and alertness concerning its polymorphic clinical features and additionally strengthen interdisciplinary approaches.

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Keywords:

POEMS syndrome, polyneuropathy, monoclonal gammopathy, diagnostic challenges

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  • Olga Vladislavovna Pirogova, MD, PhD, RM Gorbacheva Research Institute, Pavlov University, 6/8 L’va Tolstogo ul., Saint Petersburg, Russian Federation, 197022, e-mail: dr.pirogova@gmail.com

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Published

01.07.2026

Issue

Vol. 19 No. 3 (2026)
ORIGINAL ARTICLES

How to Cite

Tsynchenko A.A., Pirogova O.V., Osipov I.S., et al. Diagnostic Labyrinth of POEMS Syndrome: An Analysis of Real-World Clinical Practice. Clinical Oncohematology. Basic Research and Clinical Practice. 2026;19(3):292–301. doi:10.21320/2500-2139-2026-19-3-292-301.

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