POEMS syndrome: Polyneuropathy Organomegally Endocrinopathy M protein and Skin changes

Diagnosis:

A paraneoplastic syndrome to osteoclastic plasmacytoma

Pathology:

Muscle actin stain; increased vessels in nerves
VEGF: elevated serum levels

Criteria/Features:

Polyneuropathy: Demyelinating sensorimotor polyneuropathy (motor>sensory), usually painless, length dependent
Monoclonal gammopathy: Serum protein electrophoresis with immunofixation IFE: M protein, Lambda light chain, immunofixation is necessary to avoid false negatives
Osteoclastic plasmacytoma i.e. note lytic
Skin changes
Endocrinopathy

Other features:

Peripheral edema

Treatment:

Corticosteroids or cyclophosphamide
Consider:

  • Irradiation of solitary osteosclerotic lesions
  • Stem cell transplantation

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