Pathophysiology

The cause of POEMS syndrome is unknown. Patients frequently have higher levels of IL-1p, tumor necrosis factor a, and IL-6 than do patients with multiple myeloma.55 Levels of vascular endothelial growth factor are also frequently increased.56 Antibodies to human her-pesvirus 8 were reported in seven of nine patients with POEMS syndrome and Castleman's disease. Six of seven patients had human herpesvirus 8 DNA sequences.57

Electromyography shows slowing of nerve conduction and severe attenuation of compound muscle action potentials.58 Distal fibrillation potentials are found on needle electromyography. Both axonal degeneration and demyelination are found in sural nerve biopsy specimens. Ono et al.59 reported a loss of demyelinated fibers and an increased frequency of axonal degeneration in teased fibers. Cerebrospinal fluid protein levels are increased in almost all patients. More than half of our patients had a cerebrospinal fluid protein value more than 100 mg/dL.60

Castleman's disease (giant cell lymph node hyper-plasia, angiofollicular lymph node hyperplasia) has also been associated with POEMS syndrome.61 In our experience, about 15% of patients with POEMS syndrome also have Castleman's disease.

Renal involvement may be a feature of POEMS syndrome. Nakamoto et al.62 reviewed 52 cases of POEMS syndrome with renal abnormalities. Approximately half of the patients had creatinine levels more than 1.5 mg/dL, and 10% required dialysis.

Pulmonary hypertension also has been noted in POEMS syndrome.63 Pulmonary hypertension developed in 5 of 20 patients with POEMS syndrome, and increased levels of IL-1p, IL-6, tumor necrosis factor a, and vascular endothelial growth factor were found in all cases.64 Five of our patients had pulmonary hypertension and restrictive lung disease; respiratory failure developed in four of them. Respiratory disease was responsible for death in 17% of our patients.

Hyperpigmentation, hypertrichosis, and hemangiomas were the major dermatologic findings in our series.52 Four patients have been described with POEMS syndrome and a violaceous skin patch overlying a solitary plasmacytoma of bone, along with enlarged regional lymph nodes. One patient had POEMS syndrome, whereas in another, POEMS syndrome developed after excision of the plasmacytoma. The authors suggested the term AESOP syndrome for adenopathy and extensive skin patch overlying a plasmacytoma.65

Forty-one thrombotic events occurred in 18 of our patients. These consisted of stroke, myocardial infarction, or Budd-Chiari syndrome. Four patients with POEMS syndrome have been reported with acute arterial thrombotic events.66

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