Treatment options for POEMS syndrome

Abstract

POEMS syndrome is a rare paraneoplastic syndrome secondary to a plasma cell dyscrasia. Recognition of the complex of a combination of peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasmaproliferative disorder, skin changes, sclerotic bone lesions, Castleman’s disease, thrombocytosis, papilledema, peripheral oedema, pleural effusions, ascites, fingernail clubbing and white nails, is the first step in effectively managing the disease. More than 95% of patients will have monoclonal lambda sclerotic plasmacytoma(s) or bone marrow infiltration. In patients with a dominant sclerotic plasmacytoma, first-line therapy should include radiation to the lesion. Retrospective analysis and personal experience would dictate that systemic therapy be considered for patients with diffuse sclerotic lesions or absence of any bone lesion, and for those who have not demonstrated stabilisation of their disease 3 – 6 months after completing radiation therapy. For those patients with diffuse disease, systemic therapy is indicated. Useful approaches include therapy with corticosteroids, low dose alkylator therapy and high dose chemotherapy with peripheral blood stem cell transplant. Until the pathogenesis is fully understood, these are the mainstays of treatment for patients with POEMS syndrome.

Keywords::

• Castleman’s disease
• monoclonal gammopathy of undetermined significance
• multiple myeloma
• paraneoplastic syndrome
• POEMS syndrome
• vascular endothelial growth factor