Case of POEMS Syndrome Associated with Bilateral Macular Detachment Resolved by Autologous Peripheral Blood Stem Cell Transplantation

Correlation between peripapillary retinal thickness and serum level of vascular endothelial growth factor in patients with POEMS syndrome

“…Okada reported that the ODO in POEMS syndrome was correlated with the serum level of VEGF as we found, and patients with ODO responded well to systemic autologous peripheral blood stem cell transplantation 20. Kaushik also suggested a possible association between plasma VEGF and ODO because the median plasma VEGF level at the initial presentation was 300 pg/mL, which was significantly higher than the median VEGF level after the ODO resolved (51 pg/mL) 8…”

“…It has also been reported that the increased vascular permeability associated with elevated VEGF serum levels may contribute to the development of ODO 4 8 10 20. This concept of microvascular hyperpermeability is supported by the presence of oedema elsewhere in the body of patients with POEMS syndrome 4…”

“…The cause of the ODO in POEMS syndrome has not been determined, but it has been suggested that an elevated ICP,7–9 11 vasculitis,18 presence of cerebrospinal fluid proteins,11 infiltrations into the optic nerve,19 or increased VEGF levels4 8 10 20 may be involved.…”

Paraneoplastic and non-paraneoplastic retinopathy and optic neuropathy: Evaluation and management

“…In addition to ODE, there are at least five cases with associated CME 52,94,110,290 and one of bilateral serous macular detachment. 209 Adjunctive testing can aid in the evaluation of these patients and augment physical examination findings. Visual field testing usually demonstrates enlargement of the blind spot, 125,291 although arcuate defects are also described.…”

Changes in subfoveal choroidal thickness and reduction of serum levels of vascular endothelial growth factor in patients with POEMS syndrome

“…There is no standardised treatment for POEMS syndrome available so far, although autologous peripheral blood stem cell transplantation and systemic anti-VEGF agents application, for example, bevacizumab, had been reported to be effective 16. Thalidomide therapy had been suggested as effective treatment for POEMS syndrome which resulted in a rapid reduction in the VEGF levels by VEGF secretion or the activity reduction 17…”