The term amyloidosis refers to a group of disorders in which protein fibrils accumulate in certain organs, disrupt their tissue architecture, and impair the function of the effected organ. The clinical manifestations and prognosis vary widely depending on the specific type of the affected protein. Immunoglobulin light-chain (AL) amyloidosis is the most common form of systemic amyloidosis, characterized by deposition of a misfolded monoclonal light-chain that is secreted from a plasma cell clone. Demonstrating amyloid deposits in a tissue biopsy stained with Congo red is mandatory for the diagnosis. Novel agents (proteasome inhibitors, immunomodulatory drugs, monoclonal antibodies, venetoclax) and autologous stem cell transplantation, used for eliminating the underlying plasma cell clone, have improved the outcome for low- and intermediate-risk patients, but the prognosis for high-risk patients is still grave. Randomized studies evaluating antibodies that target the amyloid deposits (PRONTO, VITAL) were recently stopped due to futility and currently there is an intensive search for novel treatment approaches to AL amyloidosis. Early diagnosis is of paramount importance for effective treatment and prognosis, due to the progressive nature of this disease.

1.
Rajkumar
SV
,
Gertz
MA
.
Advances in the treatment of amyloidosis
.
N Engl J Med
.
2007
Jun
;
356
(
23
):
2413
5
.
[PubMed]
0028-4793
2.
Kumar
S
,
Dispenzieri
A
,
Lacy
MQ
,
Hayman
SR
,
Buadi
FK
,
Colby
C
, et al.
Revised prognostic staging system for light chain amyloidosis incorporating cardiac biomarkers and serum free light chain measurements
.
J Clin Oncol
.
2012
Mar
;
30
(
9
):
989
95
.
[PubMed]
0732-183X
3.
Palladini
G
,
Merlini
G
.
What is new in diagnosis and management of light chain amyloidosis?
Blood
.
2016
Jul
;
128
(
2
):
159
68
.
[PubMed]
0006-4971
4.
Bhat
A
,
Selmi
C
,
Naguwa
SM
,
Cheema
GS
,
Gershwin
ME
.
Currents concepts on the immunopathology of amyloidosis
.
Clin Rev Allergy Immunol
.
2010
Apr
;
38
(
2-3
):
97
106
.
[PubMed]
1080-0549
5.
G M
, editor
. AL amyloidosis: from molecular mechanisms to targeted therapies. ASH;
2017
; Atlanta, GA.
6.
Kyle
RA
,
Linos
A
,
Beard
CM
,
Linke
RP
,
Gertz
MA
,
O’Fallon
WM
, et al.
Incidence and natural history of primary systemic amyloidosis in Olmsted County, Minnesota, 1950 through 1989
.
Blood
.
1992
Apr
;
79
(
7
):
1817
22
.
[PubMed]
0006-4971
7.
Quock
TP
,
Yan
T
,
Chang
E
,
Guthrie
S
,
Broder
MS
.
Epidemiology of AL amyloidosis: a real-world study using US claims data
.
Blood Adv
.
2018
May
;
2
(
10
):
1046
53
.
[PubMed]
2473-9529
8.
Wechalekar
AD
,
Gillmore
JD
,
Bird
J
,
Cavenagh
J
,
Hawkins
S
,
Kazmi
M
, et al.;
BCSH Committee
.
Guidelines on the management of AL amyloidosis
.
Br J Haematol
.
2015
Jan
;
168
(
2
):
186
206
.
[PubMed]
0007-1048
9.
McCausland
KL
,
White
MK
,
Guthrie
SD
,
Quock
T
,
Finkel
M
,
Lousada
I
, et al.
Light Chain (AL) Amyloidosis: The Journey to Diagnosis
.
Patient
.
2018
Apr
;
11
(
2
):
207
16
.
[PubMed]
1178-1653
10.
Sher
T
,
Hayman
SR
,
Gertz
MA
.
Treatment of primary systemic amyloidosis (AL): role of intensive and standard therapy
.
[Erratum appears in Clin Adv Hematol Oncol. 2012 Nov;10(11):766]
.
Clin Adv Hematol Oncol
.
2012
Oct
;
10
(
10
):
644
51
.
[PubMed]
1543-0790
11.
Kyle
RA
,
Gertz
MA
.
Primary systemic amyloidosis: clinical and laboratory features in 474 cases
.
Semin Hematol
.
1995
Jan
;
32
(
1
):
45
59
.
[PubMed]
0037-1963
12.
Dubrey
SW
,
Cha
K
,
Anderson
J
,
Chamarthi
B
,
Reisinger
J
,
Skinner
M
, et al.
The clinical features of immunoglobulin light-chain (AL) amyloidosis with heart involvement
.
QJM
.
1998
Feb
;
91
(
2
):
141
57
.
[PubMed]
1460-2725
13.
Matsuda
M
,
Gono
T
,
Morita
H
,
Katoh
N
,
Kodaira
M
,
Ikeda
S
.
Peripheral nerve involvement in primary systemic AL amyloidosis: a clinical and electrophysiological study
.
Eur J Neurol
.
2011
Apr
;
18
(
4
):
604
10
.
[PubMed]
1351-5101
14.
Shimazaki
C
,
Hata
H
,
Iida
S
,
Ueda
M
,
Katoh
N
,
Sekijima
Y
, et al.
Nationwide Survey of 741 Patients with Systemic Amyloid Light-chain Amyloidosis in Japan
.
Intern Med
.
2018
Jan
;
57
(
2
):
181
7
.
[PubMed]
0918-2918
15.
Palladini
G
,
Campana
C
,
Klersy
C
,
Balduini
A
,
Vadacca
G
,
Perfetti
V
, et al.
Serum N-terminal pro-brain natriuretic peptide is a sensitive marker of myocardial dysfunction in AL amyloidosis
.
Circulation
.
2003
May
;
107
(
19
):
2440
5
.
[PubMed]
0009-7322
16.
Murtagh
B
,
Hammill
SC
,
Gertz
MA
,
Kyle
RA
,
Tajik
AJ
,
Grogan
M
.
Electrocardiographic findings in primary systemic amyloidosis and biopsy-proven cardiac involvement
.
Am J Cardiol
.
2005
Feb
;
95
(
4
):
535
7
.
[PubMed]
0002-9149
17.
Bhatti
S
,
Watts
E
,
Syed
F
,
Vallurupalli
S
,
Pandey
T
,
Jambekar
K
, et al.
Clinical and prognostic utility of cardiovascular magnetic resonance imaging in myeloma patients with suspected cardiac amyloidosis
.
Eur Heart J Cardiovasc Imaging
.
2016
Sep
;
17
(
9
):
970
7
.
[PubMed]
2047-2404
18.
Vogelsberg
H
,
Mahrholdt
H
,
Deluigi
CC
,
Yilmaz
A
,
Kispert
EM
,
Greulich
S
, et al.
Cardiovascular magnetic resonance in clinically suspected cardiac amyloidosis: noninvasive imaging compared to endomyocardial biopsy
.
J Am Coll Cardiol
.
2008
Mar
;
51
(
10
):
1022
30
.
[PubMed]
0735-1097
19.
Papantoniou
V
,
Valsamaki
P
,
Kastritis
S
,
Tsiouris
S
,
Delichas
Z
,
Papantoniou
Y
, et al.
Imaging of cardiac amyloidosis by (99m)Tc-PYP scintigraphy
.
Hell J Nucl Med
.
2015
Sep-Dec
;
18
Suppl 1
:
42
50
.
[PubMed]
1790-5427
20.
Harb
SC
,
Haq
M
,
Flood
K
,
Guerrieri
A
,
Passerell
W
,
Jaber
WA
, et al.
National patterns in imaging utilization for diagnosis of cardiac amyloidosis: A focus on Tc99m-pyrophosphate scintigraphy
.
J Nucl Cardiol
.
2017
Jun
;
24
(
3
):
1094
7
.
[PubMed]
1071-3581
21.
Franck
C
,
Venerito
M
,
Weigt
J
,
Roessner
A
,
Malfertheiner
P
.
Recurrent diffuse gastric bleeding as a leading symptom of gastrointestinal AL amyloidosis
.
Z Gastroenterol
.
2017
Dec
;
55
(
12
):
1318
22
.
[PubMed]
0044-2771
22.
Hoscheit
M
,
Kamal
A
,
Cline
M
.
Gastroparesis in a Patient with Gastric AL Amyloidosis
.
Case Rep Gastroenterol
.
2018
Jun
;
12
(
2
):
317
21
.
[PubMed]
1662-0631
23.
Mumford
AD
,
O’Donnell
J
,
Gillmore
JD
,
Manning
RA
,
Hawkins
PN
,
Laffan
M
.
Bleeding symptoms and coagulation abnormalities in 337 patients with AL-amyloidosis
.
Br J Haematol
.
2000
Aug
;
110
(
2
):
454
60
.
[PubMed]
0007-1048
24.
Yood
RA
,
Skinner
M
,
Rubinow
A
,
Talarico
L
,
Cohen
AS
.
Bleeding manifestations in 100 patients with amyloidosis
.
JAMA
.
1983
Mar
;
249
(
10
):
1322
4
.
[PubMed]
0098-7484
25.
Petruzziello
F
,
Zeppa
P
,
Catalano
L
,
Cozzolino
I
,
Gargiulo
G
,
Musto
P
, et al.
Amyloid in bone marrow smears of patients affected by multiple myeloma
.
Ann Hematol
.
2010
May
;
89
(
5
):
469
74
.
[PubMed]
0939-5555
26.
Duston
MA
,
Skinner
M
,
Shirahama
T
,
Cohen
AS
.
Diagnosis of amyloidosis by abdominal fat aspiration. Analysis of four years’ experience
.
Am J Med
.
1987
Mar
;
82
(
3
):
412
4
.
[PubMed]
0002-9343
27.
Garcia
Y
,
Collins
AB
,
Stone
JR
.
Abdominal fat pad excisional biopsy for the diagnosis and typing of systemic amyloidosis
.
Hum Pathol
.
2018
Feb
;
72
:
71
9
.
[PubMed]
0046-8177
28.
Fernández de Larrea
C
,
Verga
L
,
Morbini
P
,
Klersy
C
,
Lavatelli
F
,
Foli
A
, et al.
A practical approach to the diagnosis of systemic amyloidoses
.
Blood
.
2015
Apr
;
125
(
14
):
2239
44
.
[PubMed]
0006-4971
29.
Suzuki
T
,
Kusumoto
S
,
Yamashita
T
,
Masuda
A
,
Kinoshita
S
,
Yoshida
T
, et al.
Labial salivary gland biopsy for diagnosing immunoglobulin light chain amyloidosis: a retrospective analysis
.
Ann Hematol
.
2016
Jan
;
95
(
2
):
279
85
.
[PubMed]
0939-5555
30.
Vrana
JA
,
Gamez
JD
,
Madden
BJ
,
Theis
JD
,
Bergen
HR
 3rd
,
Dogan
A
.
Classification of amyloidosis by laser microdissection and mass spectrometry-based proteomic analysis in clinical biopsy specimens
.
Blood
.
2009
Dec
;
114
(
24
):
4957
9
.
[PubMed]
0006-4971
31.
Schönland
SO
,
Hegenbart
U
,
Bochtler
T
,
Mangatter
A
,
Hansberg
M
,
Ho
AD
, et al.
Immunohistochemistry in the classification of systemic forms of amyloidosis: a systematic investigation of 117 patients
.
Blood
.
2012
Jan
;
119
(
2
):
488
93
.
[PubMed]
0006-4971
32.
Hawkins
PN
,
Lavender
JP
,
Pepys
MB
.
Evaluation of systemic amyloidosis by scintigraphy with 123I-labeled serum amyloid P component
.
N Engl J Med
.
1990
Aug
;
323
(
8
):
508
13
.
[PubMed]
0028-4793
33.
Rajkumar
SV
,
Dimopoulos
MA
,
Palumbo
A
,
Blade
J
,
Merlini
G
,
Mateos
MV
, et al.
International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma
.
Lancet Oncol
.
2014
Nov
;
15
(
12
):
e538
48
.
[PubMed]
1470-2045
34.
Biewend
ML
,
Menke
DM
,
Calamia
KT
.
The spectrum of localized amyloidosis: a case series of 20 patients and review of the literature
.
Amyloid
.
2006
Sep
;
13
(
3
):
135
42
.
[PubMed]
1350-6129
35.
Weidner
T
,
Illing
T
,
Elsner
P
.
Primary Localized Cutaneous Amyloidosis: A Systematic Treatment Review
.
Am J Clin Dermatol
.
2017
Oct
;
18
(
5
):
629
42
.
[PubMed]
1175-0561
36.
Gallivan
GJ
,
Gallivan
HK
.
Laryngeal amyloidosis causing hoarseness and airway obstruction.
Journal of voice : official journal of the Voice Foundation.
2010
;24(2):235-9.
37.
Paccalin
M
,
Hachulla
E
,
Cazalet
C
,
Tricot
L
,
Carreiro
M
,
Rubi
M
, et al.
Localized amyloidosis: a survey of 35 French cases
.
Amyloid
.
2005
Dec
;
12
(
4
):
239
45
.
[PubMed]
1350-6129
38.
Ogasawara
N
,
Kitagawa
W
,
Obayashi
K
,
Itoh
Y
,
Noda
H
,
Funaki
Y
, et al.
Solitary amyloidosis of the sigmoid colon featuring submucosal tumor caused hematochezia
.
Intern Med
.
2013
;
52
(
22
):
2523
7
.
[PubMed]
0918-2918
39.
Kourelis
TV
,
Kyle
RA
,
Dingli
D
,
Buadi
FK
,
Kumar
SK
,
Gertz
MA
, et al.
Presentation and Outcomes of Localized Immunoglobulin Light Chain Amyloidosis: The Mayo Clinic Experience
.
Mayo Clin Proc
.
2017
Jun
;
92
(
6
):
908
17
.
[PubMed]
0025-6196
40.
Javed
A
,
Canales
BK
,
Maclennan
GT
.
Bladder amyloidosis
.
J Urol
.
2010
Jun
;
183
(
6
):
2388
9
.
[PubMed]
0022-5347
41.
Mahmood
S
,
Bridoux
F
,
Venner
CP
,
Sachchithanantham
S
,
Gilbertson
JA
,
Rowczenio
D
, et al.
Natural history and outcomes in localised immunoglobulin light-chain amyloidosis: a long-term observational study
.
Lancet Haematol
.
2015
Jun
;
2
(
6
):
e241
50
.
[PubMed]
2352-3026
42.
Bochtler
T
,
Hegenbart
U
,
Cremer
FW
,
Heiss
C
,
Benner
A
,
Hose
D
, et al.
Evaluation of the cytogenetic aberration pattern in amyloid light chain amyloidosis as compared with monoclonal gammopathy of undetermined significance reveals common pathways of karyotypic instability
.
Blood
.
2008
May
;
111
(
9
):
4700
5
.
[PubMed]
0006-4971
43.
Muchtar
E
,
Dispenzieri
A
,
Kumar
SK
,
Ketterling
RP
,
Dingli
D
,
Lacy
MQ
, et al.
Interphase fluorescence in situ hybridization in untreated AL amyloidosis has an independent prognostic impact by abnormality type and treatment category
.
Leukemia
.
2017
Jul
;
31
(
7
):
1562
9
.
[PubMed]
0887-6924
44.
Bochtler
T
,
Hegenbart
U
,
Kunz
C
,
Granzow
M
,
Benner
A
,
Seckinger
A
, 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
Apr
;
33
(
12
):
1371
8
.
[PubMed]
0732-183X
45.
Bochtler
T
,
Hegenbart
U
,
Kunz
C
,
Benner
A
,
Kimmich
C
,
Seckinger
A
, et al.
Prognostic impact of cytogenetic aberrations in AL amyloidosis patients after high-dose melphalan: a long-term follow-up study
.
Blood
.
2016
Jul
;
128
(
4
):
594
602
.
[PubMed]
0006-4971
46.
Wong
SW
,
Hegenbart
U
,
Palladini
G
,
Shah
GL
,
Landau
HJ
,
Warner
M
, et al.
Outcome of Patients With Newly Diagnosed Systemic Light-Chain Amyloidosis Associated With Deletion of 17p
.
Clin Lymphoma Myeloma Leuk
.
2018
Nov
;
18
(
11
):
e493
9
.
[PubMed]
2152-2650
47.
Dispenzieri
A
,
Gertz
MA
,
Kyle
RA
,
Lacy
MQ
,
Burritt
MF
,
Therneau
TM
, et al.
Prognostication of survival using cardiac troponins and N-terminal pro-brain natriuretic peptide in patients with primary systemic amyloidosis undergoing peripheral blood stem cell transplantation
.
Blood
.
2004
Sep
;
104
(
6
):
1881
7
.
[PubMed]
0006-4971
48.
Palladini
G
,
Barassi
A
,
Klersy
C
,
Pacciolla
R
,
Milani
P
,
Sarais
G
, et al.
The combination of high-sensitivity cardiac troponin T (hs-cTnT) at presentation and changes in N-terminal natriuretic peptide type B (NT-proBNP) after chemotherapy best predicts survival in AL amyloidosis
.
Blood
.
2010
Nov
;
116
(
18
):
3426
30
.
[PubMed]
0006-4971
49.
Gertz
M
,
Lacy
M
,
Dispenzieri
A
,
Hayman
S
,
Kumar
S
,
Buadi
F
, et al.
Troponin T level as an exclusion criterion for stem cell transplantation in light-chain amyloidosis
.
Leuk Lymphoma
.
2008
Jan
;
49
(
1
):
36
41
.
[PubMed]
1042-8194
51.
Kourelis
TV
,
Kumar
SK
,
Gertz
MA
,
Lacy
MQ
,
Buadi
FK
,
Hayman
SR
, et al.
Coexistent multiple myeloma or increased bone marrow plasma cells define equally high-risk populations in patients with immunoglobulin light chain amyloidosis
.
J Clin Oncol
.
2013
Dec
;
31
(
34
):
4319
24
.
[PubMed]
0732-183X
52.
Kumar
S
,
Dispenzieri
A
,
Katzmann
JA
,
Larson
DR
,
Colby
CL
,
Lacy
MQ
, et al.
Serum immunoglobulin free light-chain measurement in primary amyloidosis: prognostic value and correlations with clinical features
.
Blood
.
2010
Dec
;
116
(
24
):
5126
9
.
[PubMed]
0006-4971
53.
Sachchithanantham
S
,
Berlanga
O
,
Alvi
A
,
Mahmood
SA
,
Lachmann
HJ
,
Gillmore
JD
, et al.
Immunoparesis defined by heavy+light chain suppression is a novel marker of long-term outcomes in cardiac AL amyloidosis
.
Br J Haematol
.
2017
Nov
;
179
(
4
):
575
85
.
[PubMed]
0007-1048
54.
Muchtar
E
,
Dispenzieri
A
,
Kumar
SK
,
Buadi
FK
,
Lacy
MQ
,
Zeldenrust
S
, et al.
Immunoparesis in newly diagnosed AL amyloidosis is a marker for response and survival
.
Leukemia
.
2017
Jan
;
31
(
1
):
92
9
.
[PubMed]
0887-6924
55.
Rubinstein
S
,
Cornell
RF
,
Du
L
,
Concepcion
B
,
Goodman
S
,
Harrell
S
, et al.
Novel pathologic scoring tools predict end-stage kidney disease in light chain (AL) amyloidosis
.
Amyloid
.
2017
Sep
;
24
(
3
):
205
11
.
[PubMed]
1350-6129
56.
Muchtar
E
,
Dispenzieri
A
,
Leung
N
,
Lacy
MQ
,
Buadi
FK
,
Dingli
D
, et al.
Depth of organ response in AL amyloidosis is associated with improved survival: grading the organ response criteria
.
Leukemia
.
2018
Oct
;
32
(
10
):
2240
9
.
[PubMed]
0887-6924
57.
Rubinow
A
,
Skinner
M
,
Cohen
AS
.
Digoxin sensitivity in amyloid cardiomyopathy
.
Circulation
.
1981
Jun
;
63
(
6
):
1285
8
.
[PubMed]
0009-7322
58.
Lin
G
,
Dispenzieri
A
,
Kyle
R
,
Grogan
M
,
Brady
PA
.
Implantable cardioverter defibrillators in patients with cardiac amyloidosis
.
J Cardiovasc Electrophysiol
.
2013
Jul
;
24
(
7
):
793
8
.
[PubMed]
1045-3873
59.
Sayed
RH
,
Rogers
D
,
Khan
F
,
Wechalekar
AD
,
Lachmann
HJ
,
Fontana
M
, et al.
A study of implanted cardiac rhythm recorders in advanced cardiac AL amyloidosis
.
Eur Heart J
.
2015
May
;
36
(
18
):
1098
105
.
[PubMed]
0195-668X
60.
Scully
MS
,
Wessman
DE
,
McKee
JM
,
Francisco
GM
,
Nayak
KR
,
Kobashigawa
JA
.
Total Artificial Heart Implantation as a Bridge to Heart Transplantation in an Active Duty Service Member With Amyloid Cardiomyopathy
.
Mil Med
.
2017
Mar
;
182
(
3
):
e1858
60
.
[PubMed]
0026-4075
61.
Sattianayagam
PT
,
Gibbs
SD
,
Pinney
JH
,
Wechalekar
AD
,
Lachmann
HJ
,
Whelan
CJ
, et al.
Solid organ transplantation in AL amyloidosis
.
Am J Transplant
.
2010
Sep
;
10
(
9
):
2124
31
.
[PubMed]
1600-6135
62.
Gillmore
JD
,
Goodman
HJ
,
Lachmann
HJ
,
Offer
M
,
Wechalekar
AD
,
Joshi
J
, et al.
Sequential heart and autologous stem cell transplantation for systemic AL amyloidosis
.
Blood
.
2006
Feb
;
107
(
3
):
1227
9
.
[PubMed]
0006-4971
63.
Lacy
MQ
,
Dispenzieri
A
,
Hayman
SR
,
Kumar
S
,
Kyle
RA
,
Rajkumar
SV
, et al.
Autologous stem cell transplant after heart transplant for light chain (Al) amyloid cardiomyopathy. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation.
2008
;27(8):823-9.
64.
Bansal
T
,
Garg
A
,
Snowden
JA
,
McKane
W
.
Defining the role of renal transplantation in the modern management of multiple myeloma and other plasma cell dyscrasias
.
Nephron Clin Pract
.
2012
;
120
(
4
):
c228
35
.
[PubMed]
1660-2110
65.
Caccialanza
R
,
Palladini
G
,
Klersy
C
,
Cereda
E
,
Bonardi
C
,
Cameletti
B
, et al.
Malnutrition at diagnosis predicts mortality in patients with systemic immunoglobulin light-chain amyloidosis independently of cardiac stage and response to treatment
.
JPEN J Parenter Enteral Nutr
.
2014
Sep
;
38
(
7
):
891
4
.
[PubMed]
0148-6071
66.
Skinner
M
,
Anderson
J
,
Simms
R
,
Falk
R
,
Wang
M
,
Libbey
C
, et al.
Treatment of 100 patients with primary amyloidosis: a randomized trial of melphalan, prednisone, and colchicine versus colchicine only
.
Am J Med
.
1996
Mar
;
100
(
3
):
290
8
.
[PubMed]
0002-9343
67.
Palladini
G
,
Russo
P
,
Nuvolone
M
,
Lavatelli
F
,
Perfetti
V
,
Obici
L
, et al.
Treatment with oral melphalan plus dexamethasone produces long-term remissions in AL amyloidosis
.
Blood
.
2007
Jul
;
110
(
2
):
787
8
.
[PubMed]
0006-4971
68.
Gertz
MA
,
Lacy
MQ
,
Lust
JA
,
Greipp
PR
,
Witzig
TE
,
Kyle
RA
.
Long-term risk of myelodysplasia in melphalan-treated patients with immunoglobulin light-chain amyloidosis
.
Haematologica
.
2008
Sep
;
93
(
9
):
1402
6
.
[PubMed]
0390-6078
69.
Palladini
G
,
Milani
P
,
Foli
A
,
Obici
L
,
Lavatelli
F
,
Nuvolone
M
, 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
Apr
;
99
(
4
):
743
50
.
[PubMed]
0390-6078
70.
Milani
P
,
Schönland
S
,
Merlini
G
,
Kimmich
C
,
Foli
A
,
Dittrich
T
, et al.
Treatment of AL amyloidosis with bendamustine: a study of 122 patients
.
Blood
.
2018
Nov
;
132
(
18
):
1988
91
.
[PubMed]
0006-4971
71.
D’Souza
A
,
Dispenzieri
A
,
Wirk
B
,
Zhang
MJ
,
Huang
J
,
Gertz
MA
, et al.
Improved Outcomes After Autologous Hematopoietic Cell Transplantation for Light Chain Amyloidosis: A Center for International Blood and Marrow Transplant Research Study
.
J Clin Oncol
.
2015
Nov
;
33
(
32
):
3741
9
.
[PubMed]
0732-183X
72.
Gertz
MA
,
Lacy
MQ
,
Dispenzieri
A
,
Kumar
SK
,
Dingli
D
,
Leung
N
, et al.
Refinement in patient selection to reduce treatment-related mortality from autologous stem cell transplantation in amyloidosis
.
Bone Marrow Transplant
.
2013
Apr
;
48
(
4
):
557
61
.
[PubMed]
0268-3369
73.
Bashir
Q
,
Langford
LA
,
Parmar
S
,
Champlin
RE
,
Qazilbash
MH
.
Primary systemic amyloid light chain amyloidosis decompensating after filgrastim-induced mobilization and stem-cell collection
.
J Clin Oncol
.
2011
Feb
;
29
(
4
):
e79
80
.
[PubMed]
0732-183X
74.
Gupta
N
,
Hanley
MJ
,
Xia
C
,
Labotka
R
,
Harvey
RD
,
Venkatakrishnan
K
.
Clinical Pharmacology of Ixazomib: The First Oral Proteasome Inhibitor
.
Clin Pharmacokinet
.
2018
Aug
.
[PubMed]
0312-5963
75.
Scott
EC
,
Heitner
SB
,
Dibb
W
,
Meyers
G
,
Smith
SD
,
Abar
F
, et al.
Induction bortezomib in Al amyloidosis followed by high dose melphalan and autologous stem cell transplantation: a single institution retrospective study
.
Clin Lymphoma Myeloma Leuk
.
2014
Oct
;
14
(
5
):
424
430.e1
.
[PubMed]
2152-2650
76.
Afrough
A
,
Saliba
RM
,
Hamdi
A
,
Honhar
M
,
Varma
A
,
Cornelison
AM
, et al.
Impact of Induction Therapy on the Outcome of Immunoglobulin Light Chain Amyloidosis after Autologous Hematopoietic Stem Cell Transplantation
.
Biol Blood Marrow Transplant
.
2018
Nov
;
24
(
11
):
2197
203
.
[PubMed]
1083-8791
77.
Jaccard
A
,
Moreau
P
,
Leblond
V
,
Leleu
X
,
Benboubker
L
,
Hermine
O
, et al.;
Myélome Autogreffe (MAG) and Intergroupe Francophone du Myélome (IFM) Intergroup
.
High-dose melphalan versus melphalan plus dexamethasone for AL amyloidosis
.
N Engl J Med
.
2007
Sep
;
357
(
11
):
1083
93
.
[PubMed]
0028-4793
78.
Gertz
MA
,
Lacy
MQ
,
Dispenzieri
A
,
Buadi
FK
,
Dingli
D
,
Hayman
SR
, et al.
Stem cell transplantation compared with melphalan plus dexamethasone in the treatment of immunoglobulin light-chain amyloidosis
.
Cancer
.
2016
Jul
;
122
(
14
):
2197
205
.
[PubMed]
0008-543X
79.
Hazenberg
BP
,
Croockewit
A
,
van der Holt
B
,
Zweegman
S
,
Bos
GM
,
Delforge
M
, et al.;
Dutch-Belgian Cooperative Trial Group for Hematology Oncology
.
Extended follow up of high-dose melphalan and autologous stem cell transplantation after vincristine, doxorubicin, dexamethasone induction in amyloid light chain amyloidosis of the prospective phase II HOVON-41 study by the Dutch-Belgian Co-operative Trial Group for Hematology Oncology
.
Haematologica
.
2015
May
;
100
(
5
):
677
82
.
[PubMed]
0390-6078
80.
Browning
S
,
Quillen
K
,
Sloan
JM
,
Doros
G
,
Sarosiek
S
,
Sanchorawala
V
.
Hematologic relapse in AL amyloidosis after high-dose melphalan and stem cell transplantation
.
Blood
.
2017
Sep
;
130
(
11
):
1383
6
.
[PubMed]
0006-4971
81.
Jelinek
T
,
Kryukova
E
,
Kufova
Z
,
Kryukov
F
,
Hajek
R
.
Proteasome inhibitors in AL amyloidosis: focus on mechanism of action and clinical activity
.
Hematol Oncol
.
2017
Dec
;
35
(
4
):
408
19
.
[PubMed]
0278-0232
82.
Kastritis
E
,
Anagnostopoulos
A
,
Roussou
M
,
Toumanidis
S
,
Pamboukas
C
,
Migkou
M
, et al.
Treatment of light chain (AL) amyloidosis with the combination of bortezomib and dexamethasone
.
Haematologica
.
2007
Oct
;
92
(
10
):
1351
8
.
[PubMed]
0390-6078
83.
Palladini
G
,
Milani
P
,
Foli
A
,
Vidus Rosin
M
,
Basset
M
,
Lavatelli
F
, et al.
Melphalan and dexamethasone with or without bortezomib in newly diagnosed AL amyloidosis: a matched case-control study on 174 patients
.
Leukemia
.
2014
Dec
;
28
(
12
):
2311
6
.
[PubMed]
0887-6924
84.
Reece
DE
,
Sanchorawala
V
,
Hegenbart
U
,
Merlini
G
,
Palladini
G
,
Fermand
JP
, et al.;
VELCADE CAN2007 Study Group
.
Weekly and twice-weekly bortezomib in patients with systemic AL amyloidosis: results of a phase 1 dose-escalation study
.
Blood
.
2009
Aug
;
114
(
8
):
1489
97
.
[PubMed]
0006-4971
85.
Mikhael
JR
,
Schuster
SR
,
Jimenez-Zepeda
VH
,
Bello
N
,
Spong
J
,
Reeder
CB
, et al.
Cyclophosphamide-bortezomib-dexamethasone (CyBorD) produces rapid and complete hematologic response in patients with AL amyloidosis
.
Blood
.
2012
May
;
119
(
19
):
4391
4
.
[PubMed]
0006-4971
86.
Palladini
G
,
Sachchithanantham
S
,
Milani
P
,
Gillmore
J
,
Foli
A
,
Lachmann
H
, et al.
A European collaborative study of cyclophosphamide, bortezomib, and dexamethasone in upfront treatment of systemic AL amyloidosis
.
Blood
.
2015
Jul
;
126
(
5
):
612
5
.
[PubMed]
0006-4971
87.
Adam
D
. Cohen ECS, Michaela Liedtke, Jonathan L. Kaufman, Heather Landau, David H. Vesole, Christina L. Gomes, Cristina Gasparetto, Suzanne Lentzsch, Michael Rosenzweig, Vaishali Sanchorawala, David D. Smith, Raymond L. Comenzo, and Brian G. M. Durie, editor A Phase I Dose-Escalation Study of Carfilzomib in Patients with Previously-Treated Systemic Light-Chain (AL) Amyloidosis. ASH;
2014
.
88.
Sanchorawala
V
,
Palladini
G
,
Kukreti
V
,
Zonder
JA
,
Cohen
AD
,
Seldin
DC
, et al.
A phase 1/2 study of the oral proteasome inhibitor ixazomib in relapsed or refractory AL amyloidosis
.
Blood
.
2017
Aug
;
130
(
5
):
597
605
.
[PubMed]
0006-4971
89.
Seldin
DC
,
Choufani
EB
,
Dember
LM
,
Wiesman
JF
,
Berk
JL
,
Falk
RH
, et al.
Tolerability and efficacy of thalidomide for the treatment of patients with light chain-associated (AL) amyloidosis
.
Clin Lymphoma
.
2003
Mar
;
3
(
4
):
241
6
.
[PubMed]
1526-9655
90.
Dispenzieri
A
,
Lacy
MQ
,
Rajkumar
SV
,
Geyer
SM
,
Witzig
TE
,
Fonseca
R
, et al.
Poor tolerance to high doses of thalidomide in patients with primary systemic amyloidosis
.
Amyloid
.
2003
Dec
;
10
(
4
):
257
61
.
[PubMed]
1350-6129
91.
Dispenzieri
A
,
Dingli
D
,
Kumar
SK
,
Rajkumar
SV
,
Lacy
MQ
,
Hayman
S
, et al.
Discordance between serum cardiac biomarker and immunoglobulin-free light-chain response in patients with immunoglobulin light-chain amyloidosis treated with immune modulatory drugs
.
Am J Hematol
.
2010
Oct
;
85
(
10
):
757
9
.
[PubMed]
0361-8609
92.
Dispenzieri
A
,
Lacy
MQ
,
Zeldenrust
SR
,
Hayman
SR
,
Kumar
SK
,
Geyer
SM
, et al.
The activity of lenalidomide with or without dexamethasone in patients with primary systemic amyloidosis
.
Blood
.
2007
Jan
;
109
(
2
):
465
70
.
[PubMed]
0006-4971
93.
Dinner
S
,
Witteles
W
,
Afghahi
A
,
Witteles
R
,
Arai
S
,
Lafayette
R
, et al.
Lenalidomide, melphalan and dexamethasone in a population of patients with immunoglobulin light chain amyloidosis with high rates of advanced cardiac involvement
.
Haematologica
.
2013
Oct
;
98
(
10
):
1593
9
.
[PubMed]
0390-6078
94.
Palladini
G
,
Milani
P
,
Foli
A
,
Basset
M
,
Russo
F
,
Perlini
S
, et al.
A phase 2 trial of pomalidomide and dexamethasone rescue treatment in patients with AL amyloidosis
.
Blood
.
2017
Apr
;
129
(
15
):
2120
3
.
[PubMed]
0006-4971
95.
Kaufman
GP
,
Schrier
SL
,
Lafayette
RA
,
Arai
S
,
Witteles
RM
,
Liedtke
M
.
Daratumumab yields rapid and deep hematologic responses in patients with heavily pretreated AL amyloidosis
.
Blood
.
2017
Aug
;
130
(
7
):
900
2
.
[PubMed]
0006-4971
96.
Abeykoon
JP
,
Dispenzieri
A
, et al., editors
. Daratumumab-based therapies in patients with AL amyloidosis. The XVIth International Symposium on Amyloidosis; March 26-29; Kumamoto, Japan
2018
; Kumamoto, Japan.
97.
Leung
N
,
Thomé
SD
,
Dispenzieri
A
.
Venetoclax induced a complete response in a patient with immunoglobulin light chain amyloidosis plateaued on cyclophosphamide, bortezomib and dexamethasone
.
Haematologica
.
2018
Mar
;
103
(
3
):
e135
7
.
[PubMed]
0390-6078
98.
Gertz
MA
,
Comenzo
R
,
Falk
RH
,
Fermand
JP
,
Hazenberg
BP
,
Hawkins
PN
, 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
Aug
;
79
(
4
):
319
28
.
[PubMed]
0361-8609
99.
Palladini
G
,
Dispenzieri
A
,
Gertz
MA
,
Kumar
S
,
Wechalekar
A
,
Hawkins
PN
, 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
Dec
;
30
(
36
):
4541
9
.
[PubMed]
0732-183X
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.