scholarly journals Light chain type predicts organ involvement and survival in AL amyloidosis patients receiving stem cell transplantation

2018 ◽  
Vol 2 (7) ◽  
pp. 769-776 ◽  
Author(s):  
M Hasib Sidiqi ◽  
Mohammed A. Aljama ◽  
Eli Muchtar ◽  
Francis K. Buadi ◽  
Rahma Warsame ◽  
...  

Key Points λ Light chain AL amyloidosis is associated with a shorter PFS and OS compared with κ. Light chain type predicts likelihood of organ involvement in AL amyloidosis.

2021 ◽  
Vol 38 (1) ◽  
Author(s):  
Shanshan Zhu ◽  
Chao Yang ◽  
Wei Li ◽  
Meilin Lin

Objectives: To investigate the value of urine immunofixation electrophoresis in prognostic evaluation of hematopoietic stem cell transplantation in patients with myeloma. Methods: Thirty-four patients with multiple myeloma admitted to Affiliated Hospital of Hebei University from November 2013 to December 2014 were included as research subjects. All patients received hematopoietic stem cell transplantation and were followed up for five years. Outcomes were evaluated according to the recovery status: complete response (CR), very good partial response (VGPR), partial response (PR), stable disease (SD), and progression disease (PD). In addition, the overall response rate (CR+VGPR) of patients was observed and their urine immunoglobulin status was measured by immunofixation electrophoresis. The Kaplan-Meier method was utilized to plot the survival curve, and the Log-rank method was adopted to analyze the relationship between CR+VGPR and PR and hematopoietic stem cell transplantation (HSCT) survival in patients with myeloma. Results: The basic clinical type of immunofixation electrophoresis was as follows: 19 cases (55.88%) of IgG, 7 cases (20.59%) of IgA, 6 cases (17.65%) of IgM, and 2 cases (5.88%) of light chain type. Outcomes: 13 cases (38.24%) of CR, 12 cases (35.29%) of VGPR, 9 cases (26.47%) of PR, and 25 cases (73.53%) of the overall response rate (CR+VGPR). Compared with IgG, CR, VGPR and PR of IgA, IgM and light chain had statistically significant differences in outcome (p<0.05), and CR+VGPR of patients with IgG was higher than that of patients with IgA, IgM and light chain type (p<0.05). Two of the 34 patients were lost to follow-up. The log-rank analysis showed that the survival rate of patients with CR+VGPR was higher than that of patients with PR (p<0.05). Patients with IgA, IgM, and light chain type had an increased number of prognostic death compared with those with IgG (p<0.05). Conclusion: Patients with IgG type myeloma are superior to those with IgA, IgM and light chain type in terms of the prognosis of hematopoietic stem cell transplantation, which has a certain clinical reference value. doi: https://doi.org/10.12669/pjms.38.1.4425 How to cite this:Zhu S, Yang C, Li W, Lin M. Application of urine immunofixation electrophoresis in prognostic evaluation of hematopoietic stem cell transplantation in patients with myeloma. Pak J Med Sci. 2022;38(1):315-319. doi: https://doi.org/10.12669/pjms.38.1.4425 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


2015 ◽  
Vol 15 ◽  
pp. e173
Author(s):  
S.K. Toprak ◽  
P. Ataca ◽  
E. Atilla ◽  
S.C. Bozdag ◽  
M.K. Yuksel ◽  
...  

Author(s):  
Eli Muchtar ◽  
Angela Dispenzieri ◽  
Shaji K. Kumar ◽  
Martha Q. Lacy ◽  
Francis K. Buadi ◽  
...  

Amyloid ◽  
2011 ◽  
Vol 18 (sup1) ◽  
pp. 130-131 ◽  
Author(s):  
H. K. Meier-Ewert ◽  
V. Sanchorawala ◽  
J. Berk ◽  
K. T. Finn ◽  
M. Skinner ◽  
...  

2018 ◽  
Vol 36 (13) ◽  
pp. 1323-1329 ◽  
Author(s):  
M Hasib Sidiqi ◽  
Mohammed A. Aljama ◽  
Francis K. Buadi ◽  
Rahma M. Warsame ◽  
Martha Q. Lacy ◽  
...  

Purpose Autologous stem-cell transplantation (ASCT) has been used in patients with immunoglobulin light chain (AL) amyloidosis for more than two decades. Early experience raised concerns regarding safety with high early-mortality rates. Patients and Methods We report 20 years of experience with ASCT for AL amyloidosis at the Mayo Clinic Rochester. In all, 672 consecutive patients receiving ASCT for AL amyloidosis were divided into three cohorts on the basis of date of transplantation (cohort 1, 1996-2002 [n = 124]; cohort 2, 2003-2009 [n = 302]; and cohort 3, 2010-2016 [n = 246]). Results The median age for the entire cohort was 59 years, with patients in cohort 3 being slightly older than those in the other two cohorts (60 v 58 v 54 years for cohorts 3, 2, and 1, respectively; P < .001). Fewer patients in cohort 3 had more than two organs involved (9% v 18% v 19% for cohorts 3, 2, and 1, respectively; P < .001). More patients received pretransplantation therapy in cohort 3 compared with earlier time periods (49% v 38% v 42% for cohorts 3, 2, and 1, respectively; P = .02). Hematologic response was higher in cohort 3 (84% v 79% v 69% for cohorts 3, 2, and 1, respectively; P = .002). Median overall survival for the entire cohort was 122 months and improved over time (not reached v 120 months v 75 months for cohorts 3, 2, and 1, respectively; P < .001). Treatment-related mortality declined over time (2.4% v 8.6% v 14.5% for cohorts 3, 2, and 1, respectively; P < .001). On multivariable analysis, conditioning dose, Mayo stage 2012, and hematologic response were independent predictors of survival. Conclusion ASCT is a highly effective therapy for AL amyloidosis. The improved survival and markedly reduced treatment-related mortality in eligible patients indicate that this will remain an important first-line option even in the era of treatment approaches that use novel agents.


2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e20506-e20506
Author(s):  
Abdullah S. Al Saleh ◽  
Angela Dispenzieri ◽  
Eli Muchtar ◽  
Robert C. Wolf ◽  
David Dingli ◽  
...  

e20506 Background: Autologous stem cell transplantation (ASCT) prolongs survival in patients with light chain (AL) amyloidosis. Mayo 2012 stage and increased plasma cell percentage (%PC) are known predictors for survival. Increased beta-2 microglobulin (B2M) predicts survival in patients with multiple myeloma. However, its prognostic effect in patients with AL amyloidosis undergoing ASCT is not known. Methods: We retrospectively reviewed patients who had a diagnosis of AL amyloidosis and were treated with ASCT between July-1996 and September-2017. Patients with creatinine > 1.2 mg/dL were excluded, as that affects B2M levels. The receiver operator curve was used to determine the best cutoff for B2M in predicting survival and was 2.5 mcg/mL. Baseline characteristics were compared between patients with B2M > 2.5 and ≤2.5. Progression-free survival (PFS) was defined as time from ASCT to relapse or death, whichever occurred first. Overall survival (OS) was calculated from ASCT to death of any cause. Univariate and multivariate analysis were done for OS. Results: Five-hundred patients were identified and 222 (44%) had a B2M > 2.5. These patients were more likely to be > 65 years old (32% vs. 17%, P = 0.0001), have Mayo 2012 stage III/IV (33% vs. 8%, P < 0.0001), have ≥3 organs involved (25% vs. 14%, P = 0.001), and have ≥10% PCs (56% vs. 40%, P = 0.0002) compared to patients with B2M ≤2.5. The median PFS and OS were shorter in patients with B2M > 2.5 (median PFS: 64 vs. 80 months, P = 0.03); (median OS: 104.9 vs. 175.5 months, P < 0.0001). On univariate analysis, predictors for OS included age > 65 (HR: 1.6, P = 0.001), Mayo 2012 stage III/IV (HR: 3.3, P < 0.0001), ≥3 organs involved (HR: 1.3, P = 0.06), ≥10% PC (HR: 1.5, P = 0.004), melphalan conditioning 200mg/m2 (HR: 0.28, P < 0.0001), and B2M > 2.5 (HR: 1.8, P < 0.0001). In a multivariate analysis, only Mayo 2012 stage III/IV (HR: 1.8, P = 0.006), melphalan conditioning 200mg/m2 (HR: 0.35, P < 0.0001), and B2M > 2.5 (HR: 1.7, P = 0.01) remained independent predictive of OS. Conclusions: Beta-2 microglobulin > 2.5 is an independent predictor for OS in AL amyloidosis patients undergoing ASCT and should be routinely measured.


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