Outcome of Patients With Newly Diagnosed Systemic Light-Chain Amyloidosis Associated With Deletion of 17p Sandy W. Wong, Ute Hegenbart, Giovanni Palladini,

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Outcome of Patients With Newly Diagnosed Systemic Light-Chain Amyloidosis Associated With Deletion of 17p Sandy W. Wong, Ute Hegenbart, Giovanni Palladini, Gunjan L. Shah, Heather J. Landau, Melissa Warner, Denis Toskic, Arnaud Jaccard, Timon Hansen, Joan Bladé, M. Teresa Cibeira, Efstathios Kastritis, Angela Dispenzieri, Ashutosh Wechalekar, Cindy Varga, Stefan O. Schönland, Raymond L. Comenzo Clinical Lymphoma, Myeloma and Leukemia Volume 18, Issue 11, Pages e493-e499 (November 2018) DOI: 10.1016/j.clml.2018.07.292 Copyright © 2018 Terms and Conditions

Figure 1 Median OS and PFS. (A) OS and (B) PFS of 44 Patients With AL and del 17p at Diagnosis, Showing Median OS of 49 Months and PFS of 32 Months. (C) Patients With > 50% del 17p Marrow Plasma Cells, Although Small in Number (n = 12), Exhibited Trend Toward Shorter Survival (Median, 28 and 52 Months). (D) Survival Curve of Patients With Either t(11;14) or Gain 1q21 Exhibited Trend Toward Shorter Survival (Median, 28 and 53 Months) Abbreviations: AL = light-chain amyloidosis; OS = overall survival; PFS = progression-free survival. Clinical Lymphoma, Myeloma and Leukemia 2018 18, e493-e499DOI: (10.1016/j.clml.2018.07.292) Copyright © 2018 Terms and Conditions

Figure 2 Survival According to Treatment With Bortezomib or Alternative First-Line Therapy. (A) Survival curves of Patients Initially Treated With Either Bortezomib or Other Therapies Overlap, Indicating That Initial Therapy With Bortezomib did Not Provide Significant Survival Advantage to Patients in This Small Cohort. (B) OS of Patients Who Underwent SCT compared to Those Who did Not showed Favorable Outcomes for SCT Patients (P < .05); However, Patients Who Underwent SCT Were Younger and Had Less Advanced Cardiac Involvement. (C) Six-Month landmark Analysis of OS Based on Hematologic Response, Indicating that Patients With Responsive Disease (CR/VGPR, PR) Had Better Survival Than Those With Nonresponsive (Not Reported, NR) Disease (P < .05). (D) OS As Function of Cardiac Stage, Predictor of OS by Cox Regression Analysis (P < .05) Abbreviations: CR = complete response; OS = overall survival; PR = partial response; SCT = stem cell transplantation; VGPR = very good partial response. Clinical Lymphoma, Myeloma and Leukemia 2018 18, e493-e499DOI: (10.1016/j.clml.2018.07.292) Copyright © 2018 Terms and Conditions

Supplemental Figure S1 Clonal Evolution of Disease Into Leukemic Phase. (A) Lambda FLC, BNP, and LDH levels over course of Therapy. (B) Circulating plasma cells. (C) Clonal plasma-Cell Infiltration of lungs Abbreviations: AL = light-chain Amyloidosis; BNP = b-type Natriuretic peptide; Bor = bortezomib; BorTD = bortezomib, thalidomide, dexamethasone; CyBorD = cyclophosphamide, bortezomib, and dexamethasone; FLC = free light chain; LC = light chain; LDH = lactate dehydrogenase; VTD-PACE = multidrug regimen containing bortezomib, Thalidomide, dexamethasone, cisplatin, doxorubicin, cyclophosphamide, and etoposide. Clinical Lymphoma, Myeloma and Leukemia 2018 18, e493-e499DOI: (10.1016/j.clml.2018.07.292) Copyright © 2018 Terms and Conditions