GeriQBank · Paper III · Q17
POEMS Syndrome
- Chapter
- Haematology
- Years asked
- Not recorded in the source
- Frequency
- Set 1× in Paper IV (2009–2025)
As set in the university paper
POEMS syndrome — clinical features, laboratory investigations and treatment.
Paper IV · Oncology, Haematology & Cancer Screening · asked ×1 · 7-mark note · see in paper list
Detailed answer
Pro · free in betaAttempt it firstOutline your answer as a 7-mark note before revealing it — definition, causes, evaluation, management and the geriatric angle.Reveal answer
Complete textbook gap. Only tangential link: the standard peripheral neuropathy work-up algorithm (Essentials of Clinical Geriatrics, Fig. 13-3) includes SPEP/UPEP/IPEP for any acquired demyelinating neuropathy — the pathway that would uncover POEMS' paraproteinaemia, though the syndrome itself is never named.
High-Yield Points
POEMS = Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin changes — paraneoplastic syndrome from a low-tumour-burden, LAMBDA-restricted clonal plasma cell neoplasm.
VEGF drives the phenotype — increased vascular permeability → oedema, increased endoneural pressure, capillary leak.
Distinctive marrow: combined plasma cell, lymphoid and megakaryocytic changes.
Diagnostic Criteria (Dispenzieri, updated 2023)
Category Criteria
Mandatory major (both) Polyradiculoneuropathy + clonal plasma cell disorder (lambda-restricted) Additional major (≥1) Castleman disease, sclerotic bone lesions, elevated VEGF Minor (≥1) Organomegaly, endocrinopathy, skin changes, papilledema, extravascular volume overload, thrombocytosis Mnemonics: "POEMS" (2 mandatory + 3 minor); "PEST" (Papilledema, Extravascular overload, Sclerotic lesions, Thrombocytosis). Serum VEGF is the key diagnostic AND monitoring biomarker, independent of M-protein size.
Clinical Features
Polyradiculoneuropathy — symmetric, ascending, demyelinating — mimics CIDP (commonest cause of diagnostic delay).
Extravascular volume overload (oedema, effusions, ascites) and thrombocytosis distinguish it from ordinary CIDP.
Skin: hyperpigmentation, hypertrichosis, glomeruloid haemangiomata (near-pathognomonic).
Also: restrictive lung disease, pulmonary hypertension, increased stroke/thrombosis risk.
Untreated → death from neuropathic exhaustion or cardiopulmonary failure.
Management
Disease extent First-line approach
Localised (≤2 lesions, no marrow involvement) Radiotherapy 30–54 Gy ± lenalidomide-dexamethasone; ~82% cure Disseminated, transplant-eligible Induction chemo → autologous stem cell transplant (ASCT) — best CR/PFS rates Transplant-ineligible Lenalidomide + dexamethasone, or daratumumab + Len + dex (Dara-Rd) Rapidly deteriorating Corticosteroids as a bridge IVIg gives only short-term benefit — reliance on it (as if treating CIDP) is a recognised trap that delays correct diagnosis. Anti-VEGF therapy (bevacizumab) proved largely ineffective.
Latest Updates (2023–Present)
Diagnostic criteria formally updated (Dispenzieri, 2023). "Capillary leak phenotype" identified (Leukemia, Dec 2024) as an independent major cause of death. Daratumumab-based regimens (Dara-Rd) emerging for transplant-ineligible patients, including reports in patients in their 70s. First CAR-T and BCMA×CD3 bispecific trials for relapsed POEMS opened 2024–2025. Comprehensive 2025 ASH review (Dispenzieri) is now the reference source.
Current Practice Update
Domain Pathy's Current evidence
Coverage Not mentioned at all Full Mayo Clinic diagnostic/management framework MD Geriatrics – Haematology Compendium Page 32 of 34 Mortality mechanism Not addressed Capillary leak phenotype now recognised (2024) Transplant-ineligible therapy Not addressed Daratumumab-based triplets — relevant to older patients MD Geriatrics – Haematology Compendium Page 33 of 34
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References
- PAPERS-2-3-4_COMBINED.pdf — page 871
- GeriQBank source PDF — p. 871
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