PMID- 36974438
OWN - NLM
STAT- MEDLINE
DCOM- 20231023
LR  - 20231023
IS  - 1099-1069 (Electronic)
IS  - 0278-0232 (Linking)
VI  - 41
IP  - 4
DP  - 2023 Oct
TI  - Outcomes for patients with systemic light chain amyloidosis and Mayo stage 3B 
      disease.
PG  - 725-732
LID - 10.1002/hon.3135 [doi]
AB  - Patients with cardiac light chain amyloidosis and Mayo stage 3b disease define a 
      high-risk population with very poor prognosis. Here, we report treatment outcomes 
      of 80 consecutive patients with newly diagnosed AL and Mayo 3b who received novel 
      regimens. Early mortality (<1 month) rate was 12.5%. On intention-to-treat, 
      overall hematologic response rate was 40%, with complete response (CR)/very good 
      partial response (VGPR) in 25% and partial response (PR) in 15%. At 1- and 3- 
      month landmark analysis CR or VGPR/PR rates were 25%/23.5% and 34%/25.5%, 
      respectively. Among patients that were treated with daratumumab-based therapies, 
      52.6% and 85.7% achieved at least VGPR within one 1 and 3 months, respectively. 
      Three-month cardiac response rate was 11.3% and 6-month was 18.8%. At least 
      hemVGPR at 3 months was associated with cardiac response at 6 months (p = 0.034). 
      Median overall survival (OS) was 6.3 months. At 1-month landmark at least hemPR 
      was associated with better median OS (24.1 vs. 4.9 months, p = 0.017) and at 
      3-month landmark, at least hemVGPR was associated with a median OS of 40.7 versus 
      17 months for hemPR and 7.4 months for those without hematologic response 
      (p = 0.028). Cardiac response at 3 months was associated with longer median OS 
      (59.7 vs. 10.9 months, p = 0.044). Factors associated with poorer survival were 
      kappa-light chain amyloidosis (median OS 2.9 vs. 7.4 months, p = 0.028), peripheral 
      nerve involvement (3.4 vs. 10.45 months, p = 0.024), systolic blood pressure 
      <90 mmHg (2 vs. 8 months, p = 0.002), baseline LVEF <55% (median OS 3.4 vs. 
      32 months, p = 0.29) and New York Heart Association (NYHA) class (2.7 months for 
      NYHA 3B-4 vs. 8 months for NYHA 2-3A, p = 0.02). Twenty-one patients (26.3%) 
      received salvage therapy and ORR was 57.1%. Median OS for patients who received 
      second line therapy was 24 months. In conclusion, patients with Mayo 3b disease 
      benefit from early hematologic response but cardiac response rates remain low.
CI  - (c) 2023 John Wiley & Sons Ltd.
FAU - Theodorakakou, Foteini
AU  - Theodorakakou F
AUID- ORCID: 0000-0001-6926-0351
AD  - Department of Clinical Therapeutics, National and Kapodistrian University of 
      Athens, School of Medicine, Athens, Greece.
FAU - Briasoulis, Alexandros
AU  - Briasoulis A
AD  - Department of Clinical Therapeutics, National and Kapodistrian University of 
      Athens, School of Medicine, Athens, Greece.
FAU - Fotiou, Despina
AU  - Fotiou D
AD  - Department of Clinical Therapeutics, National and Kapodistrian University of 
      Athens, School of Medicine, Athens, Greece.
FAU - Petropoulos, Ioannis
AU  - Petropoulos I
AD  - Department of Clinical Therapeutics, National and Kapodistrian University of 
      Athens, School of Medicine, Athens, Greece.
FAU - Georgiopoulos, Georgios
AU  - Georgiopoulos G
AD  - Department of Clinical Therapeutics, National and Kapodistrian University of 
      Athens, School of Medicine, Athens, Greece.
FAU - Lama, Niki
AU  - Lama N
AD  - 2nd Department of Radiology, Research Unit of Radiology and Medical Imaging, 
      National and Kapodistrian University of Athens, School of Medicine, Athens, 
      Greece.
FAU - Kelekis, Nikolaos
AU  - Kelekis N
AD  - 2nd Department of Radiology, Research Unit of Radiology and Medical Imaging, 
      National and Kapodistrian University of Athens, School of Medicine, Athens, 
      Greece.
FAU - Repasos, Evangelos
AU  - Repasos E
AD  - Department of Clinical Therapeutics, National and Kapodistrian University of 
      Athens, School of Medicine, Athens, Greece.
FAU - Migkou, Magdalini
AU  - Migkou M
AD  - Department of Clinical Therapeutics, National and Kapodistrian University of 
      Athens, School of Medicine, Athens, Greece.
FAU - Stamatelopoulos, Kimon
AU  - Stamatelopoulos K
AD  - Department of Clinical Therapeutics, National and Kapodistrian University of 
      Athens, School of Medicine, Athens, Greece.
FAU - Dimopoulos, Meletios A
AU  - Dimopoulos MA
AD  - Department of Clinical Therapeutics, National and Kapodistrian University of 
      Athens, School of Medicine, Athens, Greece.
FAU - Kastritis, Efstathios
AU  - Kastritis E
AD  - Department of Clinical Therapeutics, National and Kapodistrian University of 
      Athens, School of Medicine, Athens, Greece.
LA  - eng
PT  - Journal Article
DEP - 20230327
PL  - England
TA  - Hematol Oncol
JT  - Hematological oncology
JID - 8307268
RN  - 0 (Immunoglobulin kappa-Chains)
SB  - IM
MH  - Humans
MH  - *Immunoglobulin Light-chain Amyloidosis/drug therapy
MH  - *Amyloidosis/complications/diagnosis
MH  - Treatment Outcome
MH  - Prognosis
MH  - Immunoglobulin kappa-Chains
MH  - Retrospective Studies
OTO - NOTNLM
OT  - NTproBNP
OT  - cardiac amyloidosis
OT  - free light chains
OT  - mayo stage
EDAT- 2023/03/29 06:00
MHDA- 2023/10/23 00:43
CRDT- 2023/03/28 02:54
PHST- 2023/03/13 00:00 [revised]
PHST- 2022/12/12 00:00 [received]
PHST- 2023/03/17 00:00 [accepted]
PHST- 2023/10/23 00:43 [medline]
PHST- 2023/03/29 06:00 [pubmed]
PHST- 2023/03/28 02:54 [entrez]
AID - 10.1002/hon.3135 [doi]
PST - ppublish
SO  - Hematol Oncol. 2023 Oct;41(4):725-732. doi: 10.1002/hon.3135. Epub 2023 Mar 27.