POEMS Syndrome - Treatment

Treatment

There are no randomised controlled trials of treatment in POEMS syndrome, owing to its rarity. Conventional treatments for demyelinating neuropathy, such as steroids, intravenous immunoglobulin and plasma exchange, are ineffective; treatment must be aimed at the haematological disorder.

Dispenzieri et al. list numerous treatments, the effect of many of which is almost anecdotal. Prednisolone and alkylating agents are the most commonly used. The Mayo Clinic group attempted hematopoietic stem cell transplantation in sixteen patients; one patient died during the treatment, and in several others respiratory problems were unmasked in the course of the procedure. The outcome of the treatment, though, was good.

Given that VEGF plays a central role in the symptoms of POEMS syndrome, some have tried bevacizumab (Avastin), a monoclonal antibody directed against VEGF. While some reports were positive, others have reported capillary leak syndrome suspected to be the result of overly rapid lowering of VEGF levels. It therefore remains doubtful as to whether this will become part of standard treatment for POEMS syndrome.

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