Opposing neurological responses to autologous peripheral blood stem cell transplantation in two patients with POEMS syndrome

Abstract

AbstractPurposeWe present our findings in two cases of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome, one with and one without optic disc edema (ODE), after autologous peripheral blood stem cell transplantation.MethodBoth patients underwent an autologous peripheral blood stem cell transplantation. The peripapillary retinal thickness (pRT) was determined by spectral‐domain optical coherence tomography (SD‐OCT) to assess the degree of optic disc edema. The SD‐OCT examinations consisted of a 3.45 mm diameter circular scan centered on the optic disc. The pRT thickness was measured at the baseline and at 12 months after beginning the treatment. The serum level of VEGF was determined by enzyme‐linked immunosorbent assays (ELISAs) at the baseline and at 12 months after beginning the treatment. Neurological examinations, e.g., grip strength and compound muscle action potentials (cMAPs) of the upper limb, were also measured at the baseline and 12 months after the transplantation.ResultThe pRT in the case without ODE was 310 μm at the baseline. At 12 months after the transplantation, the serum levels of VEGF decreased from 7050 to 511 pg/ml, the grip strength increased from 13 kg to 19 kg, and the cMAP amplitudes increased from 4.31 mV to 7.02 mV. On the other hand, the pRT in the case with severe ODE was 1167 μm at the baseline, and it did not change after the transplantation. The serum levels of VEGF decreased from 9060 pg/ml to 99 pg/ml after the transplantation, the grip strength decreased from 32 kg to 29 kg, and the amplitudes of the cMAP decreased from 2.16 mV to 0.32 mV at 12 months after the transplantation.ConclusionThese results suggest that the baseline degree of ODE before an autologous peripheral blood stem cell transplantation might be a predictive marker on the effectiveness of the transplantation on patients with the POEMS syndrome. However, further studies with a larger sample size are needed.Keywords: POEMS syndrome, vascular endothelial growth factor (VEGF), peripapillary retinal thickness (pRT), autologous peripheral blood stem cell transplantation, predictive marker