Amyloidosis and POEMS syndrome

Abstract

Importance of the field: Treatment options for amyloidosis and polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome have rapidly increased in the past years, but many patients are diagnosed late in the disease course and do not receive state-of-the art therapy.

Areas covered in this review: Stem-cell transplantation and novel agents have widened the chemotherapy alternatives available in these disorders and combinations of novel agents with high-dose therapy further improve treatment options. This review covers the main areas of debate in the optimal treatment amyloidosis and POEMS patients, focusing on the implications for everyday clinical practice and management strategies published in the past 36 months.

What the reader will gain: Insights into treatment strategies are provided in the review. Keys to early recognition of the syndromes are reviewed.

Take-home message: With early diagnosis most patients are therapy candidates. New agents and new application of stem-cell transplantation have dramatically improved outcomes for these previously uniformly poor prognosis disorders.

Keywords::

• amyloidosis
• bortezomib
• Castleman's disease
• lenalidomide
• monoclonal gammopathy of undetermined significance
• multiple myeloma
• osteosclerotic multiple myeloma
• paraneoplastic syndrome
• POEMS syndrome
• stem-cell transplantation
• thalidomide
• vascular endothelial growth factor

Acknowledgement

A Dispenzieri and this work are supported in part by NIH grants CA125614, CA62242, CA107476, and CA111345 and the Robert A Kyle Hematologic Malignancies Fund, Mayo Clinic.

Notes

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