POEMS syndrome
POEMS; Crow-Fukase syndrome; Takatsuki syndrome; PEP syndrome
POEMS syndrome is a rare multisystem paraneoplastic disorder driven by a plasma-cell dyscrasia (usually osteosclerotic myeloma or solitary plasmacytoma) with characteristic Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes. The cutaneous features β glomeruloid haemangiomas (highly characteristic), diffuse hyperpigmentation, hypertrichosis, skin thickening, clubbing, white nails, plethora, sclerodermoid change, and lymphangiectasia β are often the diagnostic clue. Median age at diagnosis 50, male predominance. Multidisciplinary haematology-led care with autologous stem-cell transplantation, lenalidomide / thalidomide, and radiotherapy for solitary plasmacytoma. Five-year survival 75β85% with modern treatment.
POEMS β diagnostic criteria
- POEMS diagnostic criteria (Dispenzieri 2017):
- Major mandatory β Polyneuropathy and Monoclonal plasma-cell disorder.
- Major (β₯ 1 required) β sclerotic bone lesion(s), Castleman disease, elevated serum VEGF.
- Minor (β₯ 1) β Organomegaly (hepatosplenomegaly, lymphadenopathy), extravascular volume overload (oedema, effusions, ascites), endocrinopathy (gonadal, adrenal, thyroid, pituitary, pancreas), skin changes, papilloedema, thrombocytosis / polycythaemia.
- Both major mandatory + 1 major + 1 minor needed for diagnosis.
- The acronym POEMS captures only some features β VEGF elevation and Castleman disease are now considered key.
Skin features
- Glomeruloid haemangiomas β pathognomonic; firm dome-shaped red-purple papules, 2β5 mm, on the trunk and extremities. Histology β capillary loops within ectatic vessels resembling renal glomeruli.
- Diffuse skin hyperpigmentation.
- Hypertrichosis (especially face).
- Skin thickening and induration (sclerodermoid).
- White nails (leuconychia totalis).
- Plethora / red facial complexion.
- Acrocyanosis.
- Clubbing.
- Lymphangiectasia, telangiectasia.
Workup
- Confirmation of plasma-cell disorder β serum protein electrophoresis, immunofixation (typically Ξ» light chain).
- Serum VEGF β elevated; supportive.
- Skeletal survey / CT β sclerotic or mixed bone lesions.
- FDG-PET-CT β Castleman disease, plasmacytoma localisation.
- Bone marrow biopsy β typically < 10% plasma cells (low burden).
- EMG / nerve-conduction studies β demyelinating + axonal polyneuropathy.
- Endocrine workup β gonadal, adrenal, thyroid, pancreas, pituitary.
- Echo + abdominal imaging β organomegaly, effusions.
- Ophthalmology β papilloedema.
Management
- Localised disease (1β3 sclerotic plasmacytomas, no bone-marrow involvement) β radiotherapy (40β50 Gy) curative in many.
- Disseminated disease β autologous stem-cell transplantation in fit patients; high response rates.
- Lenalidomide + dexamethasone β for transplant-ineligible / relapsed.
- Thalidomide + dexamethasone, bortezomib-based regimens, cyclophosphamide-based regimens β alternatives.
- VEGF inhibition (bevacizumab) β limited evidence; adjunctive.
- Long-term follow-up β neuropathy, endocrine surveillance, recurrence monitoring.
- Multidisciplinary β haematology, neurology, endocrinology, dermatology.
References
- Dispenzieri A. POEMS syndrome β 2017 update on diagnosis, risk stratification, and management. Am J Hematol; 2017.
- Bardwick PA et al. Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes. Medicine; 1980 (POEMS acronym coined).
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