Abstract
A 37-year old woman with HIV on antiretrovirals (undetectable viral load; CD4 count 520) presented with a painful length dependant sensorimotor polyneuropathy. Investigations showed mild neutropenia, IgA 5.2g/L without paraprotein. Nerve conduction studies indicated a demyelinating polyneuropathy. CSF was acellular with protein 1193mg/L. Despite multiple courses of IVIg and subsequently pulsed IV and oral steroids, her power deteriorated considerably. A nerve biopsy showed loss of myelinated fibres with inflammation. On reinvestigation an IgA lambda paraprotein (5.2g/L) with raised VEGF (1190ng/L) was identified. Bone marrow demonstrated 8% plasma cells; a CT-PET was normal. A diagnosis of POEMS syndrome was made and chemotherapy commenced. A full VEGF response and resolution of neuropathic pain was seen 6-months post treatment initiation; however, treatment was complicated by venous sinus thrombosis and hypocortisolaemia.POEMS syndrome is a rare plasma cell dyscrasia with protean clinical manifestations, though all develop a progressive peripheral neuropathy. There is no reported association with HIV and POEMS, though HIV in association with CIDP has been reported and is usually responsive to treatment. It is important to challenge a CIDP diagnosis when refractory to treatment with atypical features, particularly severe neuropathic pain, and to repeat investigations if clinical suspicion is high.liamcarroll@nhs.net
Published Version
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call