POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, & Skin changes); Crow-Fukase syndrome
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Etiology
Epidemiology
- rare
Pathology
Clinical manifestations
- demyelinating sensorimotor polyneuropathy
- ascending muscle weakness[2] (one case)
- diminished reflexes[2] (one case)
- organomegaly (hepatosplenomegaly)
- endocrinopathy
- monoclonal gammopathy
- skin & skin appendage changes
- hyperpigmentation
- hypertrichosis
- pitting edema[2] (one case)
- multicentric Castleman's disease
- osteosclerosis
- development over a 5 month period[2] (one case)
Diagnostic criteria
- diagnosis of which requires evidence of a demyeliniating polyneuropathy & a lambda monoclonal gammopathy, plus at least one other major criterion & one minor criterion
- major criteria
- minor criteria
Laboratory
- thyroid function tests
- serum protein electrophoresis (paraproteinemia)
- plasma VEGF markedly elevated[2]
- serum testosterone may be low[3]
- bone marrow biopsy[2]
- interstitial plasmacytosis consistent with plasma-cell dyscrasia
- lumbar puncture: CSF analysis
- fat pad biopsy: no evidence of amyloidosis[2]
Diagnostic procedures
- nerve conduction studies[2] (one case)
- reduced sensory & motor-nerve conduction velocities
- prolonged F responses
- electromyography[2] (one case)
- recruitment with abnormal spontaneous activity in the distal muscles of the lower extremities
- ophthalmoscopy
- optic disks were diffusely swollen[2] (one case)
Radiology
- MRI of the spine
- no evidence of mass lesion[2] (one case)
- MRI of the brain: normal[2] (one case)
- abdominal ultrasound: hepatosplenomegaly
- echocardiogram[2] (one case)
Complications
- independent risk factor for stroke
- cerebral arterial thromboses[1]
- lower limb peripheral arterial thrombosis
Management
- patients with 1-3 bone lesions & no clonal plasma cells on bone marrow biopsy, radiation therapy 45 Gy is preferred[4]
- oral glucocorticoid therapy with high-dose dexamethasone
- bortezomib in combination with dexamethasone & cyclophosphamide[4]
- lenalidomide in combination dexamethasone[4]
- daratumumab or ixazomib in combination with lenalidomide & dexamethasone[4]
- referral for stem cell transplantation
- high-dose melphalan conditioning
- autologous stem-cell transplantation[2] (one case)
Notes
- Review article June 2021[4]
More general terms
References
- ↑ 1.0 1.1 1.2 Dupont SA et al Cerebral infarction in POEMS syndrome: Incidence, risk factors, and imaging characteristics. Neurology 2009 Oct 20; 73:1308. PMID: https://www.ncbi.nlm.nih.gov/pubmed/19841383
- ↑ 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 2.11 2.12 2.13 2.14 2.15 Smith C et al Clinical problem-solving. Diagnosing One Letter at a Time. N Engl J Med 2015; 372:67-73. January 1, 2015 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/25551528 <Internet> http://www.nejm.org/doi/full/10.1056/NEJMcps1308732
- ↑ 3.0 3.1 Medical Knowledge Self Assessment Program (MKSAP) 17, 18. American College of Physicians, Philadelphia 2015, 2018
- ↑ 4.0 4.1 4.2 4.3 4.4 4.5 4.6 4.7 Khouri J, Nakashima M, Wong S Update on the Diagnosis and Treatment of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes) Syndrome. A Review. JAMA Oncol. Published online June 3, 2021 PMID: https://www.ncbi.nlm.nih.gov/pubmed/34081097 https://jamanetwork.com/journals/jamaoncology/fullarticle/2780474