The treatment of patients with systemic light chain (AL) amyloidosis is a challenge to hematologists. Despite its generally small size, the underlying clone causes a rapidly progressing, often devastating multiorgan dysfunction through the toxic light chains that form amyloid deposits. Clinical manifestations are deceitful and too often recognized at an irreversible stage. However, hematologists are in the unique position to diagnose AL amyloidosis at a presymptomatic stage, checking biomarkers of amyloid organ involvement in patients with monoclonal gammopathies at higher risk to develop the disease. Adequate technology and expertise are needed for a prompt and correct diagnosis, particularly for ruling out non-AL amyloidoses that are now also treatable. Therapy should be carefully tailored based on severity of organ involvement and clonal characteristics, and early and continual monitoring of response is critical. Three recent randomized clinical trials moved AL amyloidosis to evidence-based era. Above all, the daratumumab-bortezomib combination is a new standard-of-care for newly diagnosed patients, inducing rapid and deep responses that translate into high rates of organ response. The availability of new effective drugs allows to better personalize the therapy, reduce toxicity, and improve outcomes. Patients should be treated within clinical trials whenever possible.

1.
Merlini
G
,
Dispenzieri
A
,
Sanchorawala
V
, et al
.
Systemic immunoglobulin light chain amyloidosis
.
Nat Rev Dis Primers.
2018
;
4
(
1
):
38
.
2.
Palladini
G
,
Milani
P
,
Merlini
G
.
Management of AL amyloidosis in 2020
.
Hematology (Am Soc Hematol Educ Program).
2020
;
2020
(
1
):
363
-
371
.
3.
Brenner
DA
,
Jain
M
,
Pimentel
DR
, et al
.
Human amyloidogenic light chains directly impair cardiomyocyte function through an increase in cellular oxidant stress
.
Circ Res.
2004
;
94
(
8
):
1008
-
1010
.
4.
Lavatelli
F
,
Imperlini
E
,
Orrù
S
, et al
.
Novel mitochondrial protein interactors of immunoglobulin light chains causing heart amyloidosis
.
FASEB J.
2015
;
29
(
11
):
4614
-
4628
.
5.
Shi
J
,
Guan
J
,
Jiang
B
, et al
.
Amyloidogenic light chains induce cardiomyocyte contractile dysfunction and apoptosis via a non-canonical p38alpha MAPK pathway
.
Proc Natl Acad Sci USA.
2010
;
107
(
9
):
4188
-
4193
.
6.
Merlini
G
,
Lousada
I
,
Ando
Y
, et al
.
Rationale, application and clinical qualification for NT-proBNP as a surrogate end point in pivotal clinical trials in patients with AL amyloidosis
.
Leukemia.
2016
;
30
(
10
):
1979
-
1986
.
7.
Marin-Argany
M
,
Lin
Y
,
Misra
P
, et al
.
Cell damage in light chain amyloidosis: fibril internalization, toxicity and cell-mediated seeding
.
J Biol Chem.
2016
;
291
(
38
):
19813
-
19825
.
8.
Comenzo
RL
,
Zhang
Y
.
Ig V-L germline gene use and plasma cell burden in AL amyloidosis (AL) contribute to the tropism of organ-system involvement
.
Blood.
2000
;
96
(
11
):
154A
.
9.
Perfetti
V
,
Palladini
G
,
Casarini
S
, et al
.
The repertoire of λ light chains causing predominant amyloid heart involvement and identification of a preferentially involved germline gene, IGLV1-44
.
Blood.
2012
;
119
(
1
):
144
-
150
.
10.
Abraham
RS
,
Geyer
SM
,
Price-Troska
TL
, et al
.
Immunoglobulin light chain variable (V) region genes influence clinical presentation and outcome in light chain-associated amyloidosis (AL)
.
Blood.
2003
;
101
(
10
):
3801
-
3808
.
11.
Kourelis
TV
,
Dasari
S
,
Theis
JD
, et al
.
Clarifying immunoglobulin gene usage in systemic and localized immunoglobulin light-chain amyloidosis by mass spectrometry
.
Blood.
2017
;
129
(
3
):
299
-
306
.
12.
Perfetti
V
,
Casarini
S
,
Palladini
G
, et al
.
Analysis of V(lambda)-J(lambda) expression in plasma cells from primary (AL) amyloidosis and normal bone marrow identifies 3r (lambdaIII) as a new amyloid-associated germline gene segment
.
Blood.
2002
;
100
(
3
):
948
-
953
.
13.
Oberti
L
,
Rognoni
P
,
Barbiroli
A
, et al
.
Concurrent structural and biophysical traits link with immunoglobulin light chains amyloid propensity
.
Sci Rep.
2017
;
7
(
1
):
16809
.
14.
Morgan
GJ
,
Kelly
JW
.
The kinetic stability of a full-length antibody light chain dimer determines whether endoproteolysis can release amyloidogenic variable domains
.
J Mol Biol.
2016
;
428
(
21
):
4280
-
4297
.
15.
Blancas-Mejía
LM
,
Tischer
A
,
Thompson
JR
, et al
.
Kinetic control in protein folding for light chain amyloidosis and the differential effects of somatic mutations
.
J Mol Biol.
2014
;
426
(
2
):
347
-
361
.
16.
Maritan
M
,
Romeo
M
,
Oberti
L
, et al
.
inherent biophysical properties modulate the toxicity of soluble amyloidogenic light chains
.
J Mol Biol.
2020
;
432
(
4
):
845
-
860
.
17.
Morgan
GJ
,
Yan
NL
,
Mortenson
DE
, et al
.
Stabilization of amyloidogenic immunoglobulin light chains by small molecules
.
Proc Natl Acad Sci USA.
2019
;
116
(
17
):
8360
-
8369
.
18.
Yan
NL
,
Santos-Martins
D
,
Nair
R
, et al
.
Discovery of potent coumarin-based kinetic stabilizers of amyloidogenic immunoglobulin light chains using structure-based design
.
J Med Chem.
2021
;
64
(
9
):
6273
-
6299
.
19.
Kumar
S
,
Murray
D
,
Dasari
S
, et al
.
Assay to rapidly screen for immunoglobulin light chain glycosylation: a potential path to earlier AL diagnosis for a subset of patients [correction published in Leukemia. 2019;33:1060]
.
Leukemia.
2019
;
33
(
1
):
254
-
257
.
20.
Milani
P
,
Murray
DL
,
Barnidge
DR
, et al
.
The utility of MASS-FIX to detect and monitor monoclonal proteins in the clinic
.
Am J Hematol.
2017
;
92
(
8
):
772
-
779
.
21.
Garofalo
M
,
Piccoli
L
,
Romeo
M
, et al
.
Machine learning analyses of antibody somatic mutations predict immunoglobulin light chain toxicity
.
Nat Commun.
2021
;
12
(
1
):
3532
.
22.
Zhou
P
,
Kugelmass
A
,
Toskic
D
, et al
.
Seeking light-chain amyloidosis very early: the SAVE trial identifying clonal lambda light chain genes in patients with MGUS or smoldering multiple myeloma
.
J Clin Oncol.
2019
;
37
(
15
):
8010
.
23.
Merlini
G
,
Stone
MJ
.
Dangerous small B-cell clones
.
Blood.
2006
;
108
(
8
):
2520
-
2530
.
24.
Oliva
L
,
Orfanelli
U
,
Resnati
M
, et al
.
The amyloidogenic light chain is a stressor that sensitizes plasma cells to proteasome inhibitor toxicity
.
Blood.
2017
;
129
(
15
):
2132
-
2142
.
25.
Merlini
G
.
CyBorD: stellar response rates in AL amyloidosis
.
Blood.
2012
;
119
(
19
):
4343
-
4345
.
26.
Bochtler
T
,
Hegenbart
U
,
Kunz
C
, et al
.
Translocation t(11;14) is associated with adverse outcome in patients with newly diagnosed AL amyloidosis when treated with bortezomib-based regimens
.
J Clin Oncol.
2015
;
33
(
12
):
1371
-
1378
.
27.
Premkumar
VJ
,
Lentzsch
S
,
Pan
S
, et al
.
Venetoclax induces deep hematologic remissions in t(11;14) relapsed/refractory AL amyloidosis
.
Blood Cancer J.
2021
;
11
(
1
):
10
.
28.
Bochtler
T
,
Hegenbart
U
,
Kunz
C
, et al
.
Gain of chromosome 1q21 is an independent adverse prognostic factor in light chain amyloidosis patients treated with melphalan/dexamethasone
.
Amyloid.
2014
;
21
(
1
):
9
-
17
.
29.
Bochtler
T
,
Merz
M
,
Hielscher
T
, et al
.
Cytogenetic intraclonal heterogeneity of plasma cell dyscrasia in AL amyloidosis as compared with multiple myeloma
.
Blood Adv.
2018
;
2
(
20
):
2607
-
2618
.
30.
Alameda
D
,
Goicoechea
I
,
Vicari
M
, et al
.
Tumor cells in light-chain amyloidosis and myeloma show different transcriptional rewiring of normal plasma cell development
.
Blood.
2021
;
blood.2020009754
.
31.
Dispenzieri
A
,
Gertz
MA
,
Kyle
RA
, et al
.
Serum cardiac troponins and N-terminal pro-brain natriuretic peptide: a staging system for primary systemic amyloidosis
.
J Clin Oncol.
2004
;
22
(
18
):
3751
-
3757
.
32.
Kourelis
TV
,
Kumar
SK
,
Go
RS
, et al
.
Immunoglobulin light chain amyloidosis is diagnosed late in patients with preexisting plasma cell dyscrasias
.
Am J Hematol.
2014
;
89
(
11
):
1051
-
1054
.
33.
Schulman
A
,
Connors
LH
,
Weinberg
J
, et al
.
Patient outcomes in light chain (AL) amyloidosis: The clock is ticking from symptoms to diagnosis
.
Eur J Haematol.
2020
;
105
(
4
):
495
-
501
.
34.
Merlini
G
,
Palladini
G
.
Differential diagnosis of monoclonal gammopathy of undetermined significance
.
Hematology (Am Soc Hematol Educ Program).
2012
;
2012
(
1
):
595
-
603
.
35.
Palladini
G
,
Sachchithanantham
S
,
Milani
P
, et al
.
A European collaborative study of cyclophosphamide, bortezomib, and dexamethasone in upfront treatment of systemic AL amyloidosis
.
Blood.
2015
;
126
(
5
):
612
-
615
.
36.
Lilleness
B
,
Ruberg
FL
,
Mussinelli
R
,
Doros
G
,
Sanchorawala
V
.
Development and validation of a survival staging system incorporating BNP in patients with light chain amyloidosis
.
Blood.
2019
;
133
(
3
):
215
-
223
.
37.
Kumar
S
,
Dispenzieri
A
,
Lacy
MQ
, et al
.
Revised prognostic staging system for light chain amyloidosis incorporating cardiac biomarkers and serum free light chain measurements
.
J Clin Oncol.
2012
;
30
(
9
):
989
-
995
.
38.
Palladini
G
,
Milani
P
,
Foli
A
, et al
.
Oral melphalan and dexamethasone grants extended survival with minimal toxicity in AL amyloidosis: long-term results of a risk-adapted approach
.
Haematologica.
2014
;
99
(
4
):
743
-
750
.
39.
Palladini
G
,
Hegenbart
U
,
Milani
P
, et al
.
A staging system for renal outcome and early markers of renal response to chemotherapy in AL amyloidosis
.
Blood.
2014
;
124
(
15
):
2325
-
2332
.
40.
Sanchorawala
V
,
Sun
F
,
Quillen
K
,
Sloan
JM
,
Berk
JL
,
Seldin
DC
.
Long-term outcome of patients with AL amyloidosis treated with high-dose melphalan and stem cell transplantation: 20-year experience
.
Blood.
2015
;
126
(
20
):
2345
-
2347
.
41.
Sidiqi
MH
,
Aljama
MA
,
Buadi
FK
, et al
.
Stem cell transplantation for light chain amyloidosis: decreased early mortality over time
.
J Clin Oncol.
2018
;
36
(
13
):
1323
-
1329
.
42.
Gertz
MA
,
Schonland
S
.
Stem cell mobilization and autologous transplant for immunoglobulin light-chain amyloidosis
.
Hematol Oncol Clin North Am.
2020
;
34
(
6
):
1133
-
1144
.
43.
Cornell
RF
,
Fraser
R
,
Costa
L
, et al
.
Bortezomib-based induction is associated with superior outcomes in light chain amyloidosis patients treated with autologous hematopoietic cell transplantation regardless of plasma cell burden
.
Transplant Cell Ther.
2021
;
27
(
3
):
264
.
44.
Kastritis
E
,
Palladini
G
,
Minnema
MC
, et al;
ANDROMEDA Trial Investigators
.
Daratumumab-based treatment for immunoglobulin light-chain amyloidosis
.
N Engl J Med.
2021
;
385
(
1
):
46
-
58
.
45.
Abdallah
N
,
Sidana
S
,
Dispenzieri
A
, et al
.
Outcomes with early vs. deferred stem cell transplantation in light chain amyloidosis
.
Bone Marrow Transplant.
2020
;
55
(
7
):
1297
-
1304
.
46.
Basset
M
,
Milani
P
,
Nuvolone
M
, et al
.
Sequential response-driven bortezomib-based therapy followed by autologous stem cell transplant in AL amyloidosis
.
Blood Adv.
2020
;
4
(
17
):
4175
-
4179
.
47.
Manwani
R
,
Hegenbart
U
,
Mahmood
S
, et al
.
Deferred autologous stem cell transplantation in systemic AL amyloidosis
.
Blood Cancer J.
2018
;
8
(
11
):
101
.
48.
Palladini
G
,
Schönland
S
,
Merlini
G
, et al
.
Real-world data on patient characteristics and treatment patterns for 3000 patients with systemic AL amyloidosis in Europe between 2011 and 2018: a retrospective study by the European Myeloma Network
.
EHA Library.
2021
;
8
:
324734
.
49.
Palladini
G
,
Paiva
B
,
Wechalekar
A
, et al
.
Minimal residual disease negativity by next-generation flow cytometry is associated with improved organ response in AL amyloidosis
.
Blood Cancer J.
2021
;
11
(
2
):
34
.
50.
Muchtar
E
,
Dispenzieri
A
,
Leung
N
, et al
.
Depth of organ response in AL amyloidosis is associated with improved survival: grading the organ response criteria
.
Leukemia.
2018
;
32
(
10
):
2240
-
2249
.
51.
Palladini
G
,
Dispenzieri
A
,
Gertz
MA
, et al
.
New criteria for response to treatment in immunoglobulin light chain amyloidosis based on free light chain measurement and cardiac biomarkers: impact on survival outcomes
.
J Clin Oncol.
2012
;
30
(
36
):
4541
-
4549
.
52.
Gertz
MA
,
Comenzo
R
,
Falk
RH
, et al
.
Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL): a consensus opinion from the 10th International Symposium on Amyloid and Amyloidosis, Tours, France, 18-22 April 2004
.
Am J Hematol.
2005
;
79
(
4
):
319
-
328
.
53.
Milani
P
,
Basset
M
,
Russo
F
,
Foli
A
,
Merlini
G
,
Palladini
G
.
Patients with light-chain amyloidosis and low free light-chain burden have distinct clinical features and outcome
.
Blood.
2017
;
130
(
5
):
625
-
631
.
54.
Dittrich
T
,
Bochtler
T
,
Kimmich
C
, et al
.
AL amyloidosis patients with low amyloidogenic free light chain levels at first diagnosis have an excellent prognosis
.
Blood.
2017
;
130
(
5
):
632
-
642
.
55.
Goto
S
,
Mahara
K
,
Beussink-Nelson
L
, et al
.
Artificial intelligence-enabled fully automated detection of cardiac amyloidosis using electrocardiograms and echocardiograms
.
Nat Commun.
2021
;
12
(
1
):
2726
.
56.
Palladini
G
,
Schönland
S
,
Merlini
G
, et al
.
First glimpse on real-world efficacy outcomes for 2000 patients with systemic light chain amyloidosis in europe: a retrospective observational multicenter study by the European Myeloma Network
.
Blood.
2020
;
136
(
suppl 1
):
50
-
51
.
57.
D’Souza
A
,
Osman
K
,
Costa Chase
C
,
Borham
A
,
Bruno
M
.
The hematologist’s role in amyloidosis management: disease awareness, diagnostic workup, and practice patterns
.
Blood.
2020
;
136
(
Supplement 1
):
28
-
29
.
58.
Gillmore
JD
,
Maurer
MS
,
Falk
RH
, et al
.
Nonbiopsy diagnosis of cardiac transthyretin amyloidosis
.
Circulation.
2016
;
133
(
24
):
2404
-
2412
.
59.
Rezk
T
,
Whelan
CJ
,
Lachmann
HJ
, et al
.
Role of implantable intracardiac defibrillators in patients with cardiac immunoglobulin light chain amyloidosis
.
Br J Haematol.
2018
;
182
(
1
):
145
-
148
.
60.
Abdallah
N
,
Muchtar
E
,
Dispenzieri
A
, et al
.
Coagulation abnormalities in light chain amyloidosis
.
Mayo Clin Proc.
2021
;
96
(
2
):
377
-
387
.
61.
Manwani
R
,
Foard
D
,
Mahmood
S
, et al
.
Rapid hematologic responses improve outcomes in patients with very advanced (stage IIIb) cardiac immunoglobulin light chain amyloidosis
.
Haematologica.
2018
;
103
(
4
):
e165
-
e168
.
62.
Kristen
AV
,
Kreusser
MM
,
Blum
P
, et al
.
Improved outcomes after heart transplantation for cardiac amyloidosis in the modern era
.
J Heart Lung Transplant.
2018
;
37
(
5
):
611
-
618
.
63.
Papa
R
,
Lachmann
HJ
.
Secondary, AA, amyloidosis
.
Rheum Dis Clin North Am.
2018
;
44
(
4
):
585
-
603
.
64.
Muchtar
E
,
Gertz
MA
,
Kourelis
TV
, et al
.
Bone marrow plasma cells 20% or greater discriminate presentation, response, and survival in AL amyloidosis [correction published in Leukemia. 2020;34:2819]
.
Leukemia.
2020
;
34
(
4
):
1135
-
1143
.
65.
Kimmich
CR
,
Terzer
T
,
Benner
A
, et al
.
Daratumumab for systemic AL amyloidosis: prognostic factors and adverse outcome with nephrotic-range albuminuria
.
Blood.
2020
;
135
(
18
):
1517
-
1530
.
66.
Drayson
MT
,
Bowcock
S
,
Planche
T
, et al;
TEAMM Trial Management Group and Trial Investigators
.
Levofloxacin prophylaxis in patients with newly diagnosed myeloma (TEAMM): a multicentre, double-blind, placebo-controlled, randomised, phase 3 trial
.
Lancet Oncol.
2019
;
20
(
12
):
1760
-
1772
.
67.
Roussel
M
,
Merlini
G
,
Chevret
S
, et al
.
A prospective phase 2 trial of daratumumab in patients with previously treated systemic light-chain amyloidosis
.
Blood.
2020
;
135
(
18
):
1531
-
1540
.
68.
Palladini
G
,
Kastritis
E
,
Maurer
MS
, et al
.
Daratumumab plus CyBorD for patients with newly diagnosed AL amyloidosis: safety run-in results of ANDROMEDA
.
Blood.
2020
;
136
(
1
):
71
-
80
.
69.
Muchtar
E
,
Dispenzieri
A
,
Gertz
MA
, et al
.
Treatment of AL amyloidosis: Mayo stratification of myeloma and risk-adapted therapy (mSMART) consensus statement 2020 update
.
Mayo Clin Proc.
2021
;
96
(
6
):
1546
-
1577
.
70.
Cibeira
MT
,
Ortiz-Pérez
JT
,
Quintana
LF
,
Fernádez de Larrea
C
,
Tovar
N
,
Bladé
J
.
Supportive care in AL amyloidosis
.
Acta Haematol.
2020
;
143
(
4
):
335
-
342
.
71.
Le Bras
F
,
Molinier-Frenkel
V
,
Guellich
A
, et al
.
Sequential cyclophosphamide-bortezomib-dexamethasone unmasks the harmful cardiac effect of dexamethasone in primary light-chain cardiac amyloidosis
.
Eur J Cancer.
2017
;
76
:
183
-
187
.
72.
Manwani
R
,
Cohen
O
,
Sharpley
F
, et al
.
A prospective observational study of 915 patients with systemic AL amyloidosis treated with upfront bortezomib
.
Blood.
2019
;
134
(
25
):
2271
-
2280
.
73.
Kastritis
E
,
Leleu
X
,
Arnulf
B
, et al
.
Bortezomib, melphalan, and dexamethasone for light-chain amyloidosis
.
J Clin Oncol.
2020
;
38
(
28
):
3252
-
3260
.
74.
Abdallah
N
,
Dispenzieri
A
,
Muchtar
E
, et al
.
Prognostic restaging after treatment initiation in patients with AL amyloidosis
.
Blood Adv.
2021
;
5
(
4
):
1029
-
1036
.
75.
Milani
P
,
Gertz
MA
,
Merlini
G
,
Dispenzieri
A
.
Attitudes about when and how to treat patients with AL amyloidosis: an international survey [correction published in Amyloid. 2017;24(4):256]
.
Amyloid.
2017
;
24
(
4
):
213
-
216
.
76.
Sanchorawala
V
.
Delay treatment of AL amyloidosis at relapse until symptomatic: devil is in the details
.
Blood Adv.
2019
;
3
(
2
):
216
-
218
.
77.
Palladini
G
,
Merlini
G
.
When should treatment of AL amyloidosis start at relapse? Early, to prevent organ progression
.
Blood Adv.
2019
;
3
(
2
):
212
-
215
.
78.
Hwa
YL
,
Warsame
R
,
Gertz
MA
, et al
.
Delineation of the timing of second-line therapy post-autologous stem cell transplant in patients with AL amyloidosis
.
Blood.
2017
;
130
(
13
):
1578
-
1584
.
79.
Palladini
G
,
Milani
P
,
Foli
A
, et al
.
Presentation and outcome with second-line treatment in AL amyloidosis previously sensitive to nontransplant therapies
.
Blood.
2018
;
131
(
5
):
525
-
532
.
80.
Cohen
OC
,
Sharpley
F
,
Gillmore
JD
, et al
.
Use of ixazomib, lenalidomide and dexamethasone in patients with relapsed amyloid light-chain amyloidosis
.
Br J Haematol.
2020
;
189
(
4
):
643
-
649
.
81.
Kastritis
E
,
Fotiou
D
,
Theodorakakou
F
, et al
.
Timing and impact of a deep response in the outcome of patients with systemic light chain (AL) amyloidosis
.
Amyloid.
2021
;
28
(
1
):
3
-
11
.
82.
Sidana
S
,
Milani
P
,
Binder
M
, et al
.
A validated composite organ and hematologic response model for early assessment of treatment outcomes in light chain amyloidosis
.
Blood Cancer J.
2020
;
10
(
4
):
41
.
83.
Palladini
G
,
Russo
P
,
Bosoni
T
, et al
.
AL amyloidosis associated with IgM monoclonal protein: a distinct clinical entity
.
Clin Lymphoma Myeloma.
2009
;
9
(
1
):
80
-
83
.
84.
Sachchithanantham
S
,
Roussel
M
,
Palladini
G
, et al
.
European Collaborative Study defining clinical profile outcomes and novel prognostic criteria in monoclonal immunoglobulin M-Related light chain amyloidosis
.
J Clin Oncol.
2016
;
34
(
17
):
2037
-
2045
.
85.
Sidana
S
,
Larson
DP
,
Greipp
PT
, et al
.
IgM AL amyloidosis: delineating disease biology and outcomes with clinical, genomic and bone marrow morphological features
.
Leukemia.
2020
;
34
(
5
):
1373
-
1382
.
86.
Palladini
G
,
Foli
A
,
Russo
P
, et al
.
Treatment of IgM-associated AL amyloidosis with the combination of rituximab, bortezomib, and dexamethasone
.
Clin Lymphoma Myeloma Leuk.
2011
;
11
(
1
):
143
-
145
.
87.
Milani
P
,
Schönland
S
,
Merlini
G
, et al
.
Treatment of AL amyloidosis with bendamustine: a study of 122 patients
.
Blood.
2018
;
132
(
18
):
1988
-
1991
.
88.
Lentzsch
S
,
Lagos
GG
,
Comenzo
RL
, et al
.
Bendamustine with dexamethasone in relapsed/refractory systemic light-chain amyloidosis: results of a phase II study
.
J Clin Oncol.
2020
;
38
(
13
):
1455
-
1462
.
89.
Sidiqi
MH
,
Buadi
FK
,
Dispenzieri
A
, et al
.
Autologous stem cell transplant for IgM-associated amyloid light-chain amyloidosis
.
Biol Blood Marrow Transplant.
2019
;
25
(
3
):
e108
-
e111
.
90.
Pika
T
,
Hegenbart
U
,
Flodrova
P
,
Maier
B
,
Kimmich
C
,
Schönland
SO
.
First report of ibrutinib in IgM-related amyloidosis: few responses, poor tolerability, and short survival
.
Blood.
2018
;
131
(
3
):
368
-
371
.
91.
Castillo
JJ
,
Callander
NS
,
Baljevic
M
,
Sborov
DW
,
Kumar
S
.
The evaluation and management of monoclonal gammopathy of renal significance and monoclonal gammopathy of neurological significance
.
Am J Hematol.
2021
;
96
(
7
):
846
-
853
.
92.
Dispenzieri
A
,
Kastritis
E
,
Wechalekar
AD
, et al
.
A randomized phase 3 study of ixazomib-dexamethasone versus physician’s choice in relapsed or refractory AL amyloidosis
.
Leukemia.
2021
.
93.
Parker
T
,
Rosenthal
A
,
Sanchorawala
V
, et al
.
A phase II study of isatuximab (SAR650984) (NSC-795145) for patients with previously treated AL amyloidosis (SWOG S1702; NCT#03499808)
.
Blood.
2020
;
136
(
suppl 1
):
20
-
21
.
94.
Edwards
CV
,
Rao
N
,
Bhutani
D
, et al
.
Phase 1a/b study of monoclonal antibody CAEL-101 (11-1F4) in patients with AL amyloidosis [published online ahead of print 14 September 2021]
.
Blood.
2021
95.
Ward
JE
,
Ren
R
,
Toraldo
G
, et al
.
Doxycycline reduces fibril formation in a transgenic mouse model of AL amyloidosis
.
Blood.
2011
;
118
(
25
):
6610
-
6617
.
96.
Diomede
L
,
Rognoni
P
,
Lavatelli
F
, et al
.
A Caenorhabditis elegans-based assay recognizes immunoglobulin light chains causing heart amyloidosis
.
Blood.
2014
;
123
(
23
):
3543
-
3552
.
97.
Kumar
SK
,
Dispenzieri
A
,
Lacy
MQ
, et al
.
Doxycycline used as post transplant antibacterial prophylaxis improves survival in patients with light chain amyloidosis undergoing autologous stem cell transplantation
.
Blood.
2012
;
120
(
21
):
3138
.
98.
Wechalekar
AD
,
Whelan
C
.
Encouraging impact of doxycycline on early mortality in cardiac light chain (AL) amyloidosis
.
Blood Cancer J.
2017
;
7
(
3
):
e546
.
99.
D’Souza
A
,
Szabo
A
,
Flynn
KE
, et al
.
Adjuvant doxycycline to enhance anti-amyloid effects: Results from the dual phase 2 trial
.
EClinicalMedicine.
2020
;
23
:
100361
.
100.
Shen
KN
,
Fu
WJ
,
Wu
Y
, et al
.
Doxycycline Combined With Bortezomib-Cyclophosphamide-Dexamethasone Chemotherapy for Newly Diagnosed Cardiac Light-Chain Amyloidosis: A Multicenter Randomized Controlled Trial
.
Circulation
.
2021
[Epub ahead of print].
101.
Law
S
,
Cohen
O
,
Lachmann
HJ
, et al
.
Renal transplant outcomes in amyloidosis
.
Nephrol Dial Transplant.
2021
;
36
(
2
):
355
-
365
.
102.
Dasari
S
,
Theis
JD
,
Vrana
JA
, et al
.
Amyloid typing by mass spectrometry in clinical practice: a comprehensive review of 16,175 samples
.
Mayo Clin Proc.
2020
;
95
(
9
):
1852
-
1864
.
103.
Fernández de Larrea
C
,
Verga
L
,
Morbini
P
, et al
.
A practical approach to the diagnosis of systemic amyloidoses
.
Blood.
2015
;
125
(
14
):
2239
-
2244
.
104.
Nguyen
VP
,
Landau
H
,
Quillen
K
, et al
.
Modified high-dose melphalan and autologous stem cell transplantation for immunoglobulin light chain amyloidosis
.
Biol Blood Marrow Transplant.
2018
;
24
(
9
):
1823
-
1827
.
105.
Manwani
R
,
Cohen
O
,
Sharpley
F
, et al
.
A prospective observational study of 915 patients with systemic AL amyloidosis treated with upfront bortezomib
.
Blood.
2019
;
134
(
25
):
2271
-
2280
.
106.
Landau
H
,
Hassoun
H
,
Rosenzweig
MA
, et al
.
Bortezomib and dexamethasone consolidation following risk-adapted melphalan and stem cell transplantation for patients with newly diagnosed light-chain amyloidosis
.
Leukemia.
2013
;
27
(
4
):
823
-
828
.
107.
Tandon
N
,
Sidana
S
,
Gertz
MA
, et al
.
Treatment patterns and outcome following initial relapse or refractory disease in patients with systemic light chain amyloidosis
.
Am J Hematol.
2017
;
92
(
6
):
549
-
554
.
108.
Sanchorawala
V
,
Sarosiek
S
,
Schulman
A
, et al
.
Safety, tolerability, and response rates of daratumumab in relapsed AL amyloidosis: results of a phase 2 study
.
Blood.
2020
;
135
(
18
):
1541
-
1547
.
109.
Mahmood
S
,
Venner
CP
,
Sachchithanantham
S
, et al
.
Lenalidomide and dexamethasone for systemic AL amyloidosis following prior treatment with thalidomide or bortezomib regimens
.
Br J Haematol.
2014
;
166
(
6
):
842
-
848
.
110.
Milani
P
,
Sharpley
F
,
Schönland
SO
, et al
.
Pomalidomide and dexamethasone grant rapid haematologic responses in patients with relapsed and refractory AL amyloidosis: a European retrospective series of 153 patients
.
Amyloid.
2020
;
27
(
4
):
231
-
236
.
111.
Specter
R
,
Sanchorawala
V
,
Seldin
DC
, et al
.
Kidney dysfunction during lenalidomide treatment for AL amyloidosis
.
Nephrol Dial Transplant.
2011
;
26
(
3
):
881
-
886
.
112.
Sanchorawala
V
,
Palladini
G
,
Kukreti
V
, et al
.
A phase 1/2 study of the oral proteasome inhibitor ixazomib in relapsed or refractory AL amyloidosis [correction published in Blood. 2020:135(13):1071]
.
Blood.
2017
;
130
(
5
):
597
-
605
.
You do not currently have access to this content.

Sign in via your Institution

Sign In