Light-chain Amyloidosis with Concomitant Symptomatic Myeloma (CRAB-SLiM Features): Clinical Characteristics, Cytogenetic Abnormalities, and Outcomes
Abstract Patients with light chain (AL) amyloidosis and concomitant symptomatic multiple myeloma (MM) are known to have a worse prognosis, while the prognostic implication of cytogenetic abnormalities (CA) and optimal treatment schemes are not well-established. This study retrospectively evaluated the clinical characteristics, CA, and outcomes of 106 patients with AL amyloidosis and concomitant symptomatic MM (MM-AL) by comparing with patients with MM (n=658) or primary AL amyloidosis (pAL, n=151) alone. The prevalence of t(11;14) was significantly higher in the pAL-alone group than in MM-AL and MM-alone group (40.7 % vs. 25.7% vs. 16.6%, p<0.001), and the prevalence of del13q, gain1q21 and high-risk CA (HRCA) decrease in turn in MM-alone, MM-AL and pAL-alone group. The progression-free survival (PFS) and overall survival (OS) of MM-AL patients (median, 12.8 and 25.2 months) were significantly inferior to patients with MM-alone and pAL-alone. No significant difference in PFS and OS was found between MM-AL patients with and without HRCA. When stratified by the type of plasma cell disease and status of t(11;14), patients with MM-AL and t(11;14) presented the worst OS (median, 8.2 months, p<0.001). Regarding the management of MM-AL, extended cycles of induction therapy and the use of maintenance therapy contributed to a better prognosis..
| Medienart: |
|
|---|
Preprint| Erscheinungsjahr: |
2024 |
|---|---|
| Erschienen: |
2024 |
| Enthalten in: |
ResearchSquare.com - (2024) vom: 18. März Zur Gesamtaufnahme - year:2024 |
|---|
| Sprache: |
Englisch |
|---|
| Beteiligte Personen: |
Liu, Peng [VerfasserIn] |
|---|
| Links: |
Volltext [kostenfrei] |
|---|
| Themen: |
|---|
| doi: |
10.21203/rs.3.rs-3879254/v1 |
|---|
| funding: |
|
|---|---|
| Förderinstitution / Projekttitel: |
|
| PPN (Katalog-ID): |
XRA042292018 |
|---|
| LEADER | 01000caa a22002652 4500 | ||
|---|---|---|---|
| 001 | XRA042292018 | ||
| 003 | DE-627 | ||
| 005 | 20240319131914.0 | ||
| 007 | cr uuu---uuuuu | ||
| 008 | 240126s2024 xx |||||o 00| ||eng c | ||
| 024 | 7 | |a 10.21203/rs.3.rs-3879254/v1 |2 doi | |
| 035 | |a (DE-627)XRA042292018 | ||
| 035 | |a (ResearchSquare)10.21203/rs.3.rs-3879254/v1 | ||
| 040 | |a DE-627 |b ger |c DE-627 |e rakwb | ||
| 041 | |a eng | ||
| 100 | 1 | |a Liu, Peng |e verfasserin |0 (orcid)0000-0002-9639-6606 |4 aut | |
| 245 | 1 | 0 | |a Light-chain Amyloidosis with Concomitant Symptomatic Myeloma (CRAB-SLiM Features): Clinical Characteristics, Cytogenetic Abnormalities, and Outcomes |
| 264 | 1 | |c 2024 | |
| 336 | |a Text |b txt |2 rdacontent | ||
| 337 | |a Computermedien |b c |2 rdamedia | ||
| 338 | |a Online-Ressource |b cr |2 rdacarrier | ||
| 520 | |a Abstract Patients with light chain (AL) amyloidosis and concomitant symptomatic multiple myeloma (MM) are known to have a worse prognosis, while the prognostic implication of cytogenetic abnormalities (CA) and optimal treatment schemes are not well-established. This study retrospectively evaluated the clinical characteristics, CA, and outcomes of 106 patients with AL amyloidosis and concomitant symptomatic MM (MM-AL) by comparing with patients with MM (n=658) or primary AL amyloidosis (pAL, n=151) alone. The prevalence of t(11;14) was significantly higher in the pAL-alone group than in MM-AL and MM-alone group (40.7 % vs. 25.7% vs. 16.6%, p<0.001), and the prevalence of del13q, gain1q21 and high-risk CA (HRCA) decrease in turn in MM-alone, MM-AL and pAL-alone group. The progression-free survival (PFS) and overall survival (OS) of MM-AL patients (median, 12.8 and 25.2 months) were significantly inferior to patients with MM-alone and pAL-alone. No significant difference in PFS and OS was found between MM-AL patients with and without HRCA. When stratified by the type of plasma cell disease and status of t(11;14), patients with MM-AL and t(11;14) presented the worst OS (median, 8.2 months, p<0.001). Regarding the management of MM-AL, extended cycles of induction therapy and the use of maintenance therapy contributed to a better prognosis. | ||
| 650 | 4 | |a Biology |7 (dpeaa)DE-84 | |
| 650 | 4 | |a 570 |7 (dpeaa)DE-84 | |
| 700 | 1 | |a Yu, Chenqi |4 aut | |
| 700 | 1 | |a Li, Jing |0 (orcid)0000-0001-9187-7809 |4 aut | |
| 700 | 1 | |a Xu, Tianhong |4 aut | |
| 700 | 1 | |a Wang, Wenjing |4 aut | |
| 700 | 1 | |a Yang, Yang |4 aut | |
| 700 | 1 | |a Zhou, Chi |4 aut | |
| 700 | 1 | |a Wang, Pu |4 aut | |
| 773 | 0 | 8 | |i Enthalten in |t ResearchSquare.com |g (2024) vom: 18. März |
| 773 | 1 | 8 | |g year:2024 |g day:18 |g month:03 |
| 856 | 4 | 0 | |u http://dx.doi.org/10.21203/rs.3.rs-3879254/v1 |z kostenfrei |3 Volltext |
| 912 | |a GBV_XRA | ||
| 951 | |a AR | ||
| 952 | |j 2024 |b 18 |c 03 | ||