Natural history and outcome of light chain deposition disease ... | 合法醫療器材資訊網

Lightchaindepositiondisease(LCDD)ischaracterizedbythedepositionofmonotypicimmunoglobulinlightchainsinthekidney,resultinginrenaldysfunction.

We compared renal outcomes among 32 evaluable patients who achieved different levels of clonal response (as determined by changes in their dFLC values) to first-line chemotherapy and/or autologous stem cell transplant (ASCT). The first-line chemotherapy regimens that were administered to these patients are shown in Table 2[1]. There was a mean improvement in GFR of 6.1 mL/min per year from a baseline of 26 mL/min per year among 21 patients who achieved a hematologic CR/VGPR at 6 months compared with a mean GFR loss of 6.5 mL/min per year from a baseline of 34 mL/min per year among both those who achieved a hematologic PR (n = 4) or NR (n = 7; Figure 3[2]; P < .009). Only 3 of 21 patients who achieved a hematologic CR/VGPR with first-line chemotherapy required RRT throughout follow-up compared with 7 of 11 patients who achieved an initial PR/NR (Figure 2C[3]; P = .005). Median time to hematologic relapse a...