RESUMO
Background: Late gadolinium enhancement (LGE) is a classic imaging modality derived from cardiac magnetic resonance (CMR), which is commonly used to describe cardiac tissue characterization. T1 mapping with extracellular volume (ECV) and native T1 are novel quantitative parameters. The prognostic value of multiparametric CMR in patients with light chain (AL) amyloidosis remains to be thoroughly investigated. Methods: A total of 89 subjects with AL amyloidosis were enrolled from April 2016 to January 2021, and all of them underwent CMR on a 3.0 T scanner. The clinical outcome and therapeutic effect were observed. Cox regression was used to investigate the effect of multiple CMR parameters on outcomes in this population. Results: LGE extent, native T1 and ECV correlated well with cardiac biomarkers. During a median follow-up of 40 months, 21 patients died. ECV (hazard ratio [HR]: 2.087 for per 10% increase, 95% confidence interval [CI]: 1.379-3.157, P < 0.001) and native T1 (HR: 2.443 for per 100 ms increase, 95% CI: 1.381-4.321, P=0.002) were independently predictive of mortality. A novel prognostic staging system based on median native T1 (1344 ms) and ECV (40%) was similar to Mayo 2004 Stage, and the 5-year estimated overall survival rates in Stage I, II, and III were 95%, 80%, and 53%, respectively. In patients with ECV > 40%, receiving autologous stem cell transplantation had higher cardiac and renal response rates than conventional chemotherapy. Conclusion: Both native T1 and ECV independently predict mortality in patients with AL amyloidosis. Receiving autologous stem cell transplantation is effective and significantly improves the clinical outcomes in patients with ECV > 40%.
RESUMO
Engraftment syndrome (ES) is a clinical complication that occurs during the neutrophil recovery phase following hematopoietic stem cell transplantation. The clinical features of ES in light chain (AL) amyloidosis remains to be thoroughly investigated. This study was conducted to better understand the characteristics of ES following autologous stem cell transplantation (ASCT) in AL amyloidosis with renal involvement. We conducted this single-center retrospective study in 302 patients with AL amyloidosis who underwent ASCT between July 2010 and December 2021. Sixty-seven of the 302 patients (22.2%) developed ES, with a median time to the occurrence of ES after stem cell reinfusion of 11 days (range, 7 to 17 days). Among the outcome measures in this study, estimated glomerular filtration rate (eGFR) at baseline and C-reactive protein (CRP) level on the day of granulocyte engraftment were statistically different between the ES patients and non-ES patients. We observed no significant difference between the 2 groups in transplantation-related adverse events (grade ≥ 2), hematologic and organ responses, overall survival, and progression-free survival. Furthermore, CRP level at granulocyte engraftment (odds ratio [OR], 1.012; 95% confidence interval [CI], 1.004 to 1.020; P = .002) and the absence of induction chemotherapy before ASCT (OR, 1.977; 95% CI, 1.047 to 3.731; P = .036) were identified as risk factors for the development of ES, whereas a higher eGFR at baseline (OR, .981; 95% CI, .969 to .993; P = .002) was identified as a protective factor against ES. Our data show a 22.2% incidence of ES in AL amyloidosis patients with renal involvement after ASCT and identify associated risk and protective factors, which can improve the understanding of this clinical complication.