Rates of Engraftment Syndrome after Autologous Stem Cell Transplantation in Patients with Immunoglobulin Light Chain Amyloidosis

Background Engraftment syndrome (ES) is a complication of autologous hematopoietic stem cell transplantation (auto-HCT), often characterized by non-infectious fever, rash, diarrhea, hepatic or renal dysfunction and non-cardiogenic pulmonary edema at the time of neutrophil recovery. Based on our experience observing increased incidence of ES in recent years, we conducted single center study and evaluated incidence of ES in patients (pts) with light chain amyloidosis (AL). Methods We reviewed data of 72 AL pts who underwent auto-HCT at our center between 1999 and 2017. We reviewed data regarding incidence of ES, feature of ES and evaluated it effects on outcome post auto-HCT. Engraftment syndrome was defined according to Maiolino criteria (Bone Marrow Transplant 2003; 31(5):393-7). Results Baseline characteristics are summarized in the figure 1. The median age of pts at auto-HCT was 58 years (range, 30-75). Twenty-two (31%) pts developed ES, two patients died before neutrophil engraftment. Most common symptoms observed with ES were fever (77%), diarrhea (73%), rash (68%), weight gain (56%), non-cardiogenic pulmonary edema (23%), and acute kidney injury (9%), as shown in figure 2. All patients with ES were treated with steroids. One patient with ES required hemodialysis and management in intensive care unit. In univariate analysis, age (p= 0.23), AL stage (p= 0.24), number of organs involved (p= 0.30), cytoreductive therapy prior to auto-HCT (p= 0.5), method of stem cell mobilization (G vs P+G; p= 0.16) and dose of melphalan used (p= 0.19) were not predictive of ES, as shown in figure 3. Similarly, day 100 post auto-HCT hematological response (19.5% vs 14%, p= 0.7), best organ response (23% vs 36%, p= 0.24) and median length of hospital stay after auto-HCT (20 vs 19 days, p= 0.5) were not significantly different between patients who did not or develop ES, respectively. Overall survival censored at day 180 post auto-HCT to focus on short term outcome, 100% and 80% were alive with or without ES, respectively (p= 0.3; Fig. 3). Conclusion In this single center series, we report a relatively high rate of ES among recipients of auto-HCT for AL compared with previous reports in AL (8%) (Irazabel MV et al. AJH 2012;87[1]:51–54) or with auto-HCT for MM at our center (21%) (Cornell, RF et al. Biol Blood Marrow Transplant. 2013;19[9]:1368-73). Although higher morbidity (ICU stay/ peri engraftment weight change) appeared to be associated with ES, statistically significant predictors or sequelae could not be identified. A larger series with multicenter collaboration is needed to better identify the risk factors associated with ES in AL pts.