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POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, & Skin changes); Crow-Fukase syndrome
Etiology:
- monoclonal gammopathy of uncertain significance
- multiple myeloma
- solitary plasmacytoma of bone
Epidemiology:
- rare
Pathology:
- vasculopathy (non-inflammatory) [1]
- demyelination [2]
Clinical manifestations:
- demyelinating sensorimotor polyneuropathy
- ascending muscle weakness [2] (one case)
- diminished reflexes [2] (one case)
- organomegaly (hepatosplenomegaly)
- endocrinopathy
- hypogonadism
- adrenal insufficiency
- hypothyroidism
- diabetes mellitus
- 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
- sclerotic bone lesion
- elevated plasma VEGF level
- Castleman's disease (angiofollicular lymph node hyperplasia) [4]
- minor criteria
- organomegaly
- fluid overload
- papilledema
- endocrinopathy: hypothyroidism, diabetes mellitus
- skin changes
- thrombocytosis & polycythemia
- papilledema [4]
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]
Special laboratory:
- 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)
- pulmonary hypertension
- right ventricular hypertrophy
- tricuspid regurgitation
- pericardial effusion
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]
Interactions
disease interactions
General
paraproteinemia (monoclonal gammopathy)
peripheral nerve disease; peripheral neuropathy
References
- Dupont SA et al
Cerebral infarction in POEMS syndrome: Incidence, risk factors,
and imaging characteristics.
Neurology 2009 Oct 20; 73:1308.
PMID: 19841383
- Smith C et al
Clinical problem-solving. Diagnosing One Letter at a Time.
N Engl J Med 2015; 372:67-73. January 1, 2015
PMID: 25551528
http://www.nejm.org/doi/full/10.1056/NEJMcps1308732
- Medical Knowledge Self Assessment Program (MKSAP) 17, 18.
American College of Physicians, Philadelphia 2015, 2018
- 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: 34081097
https://jamanetwork.com/journals/jamaoncology/fullarticle/2780474