Immunoglobulin light-chain amyloidosis: From basics to new developments in diagnosis, prognosis and therapy

Acta Haematologica

Volumn 135, Issue 3, 2016, Pages 172-190

  Muchtar, Eli ^a   Buadi, Francis K ^a   Dispenzieri, Angela ^a   Gertz, Morie A ^a  

^a MAYO CLINIC   (United States)

Author keywords

Diagnosis; Light chain amyloidosis; Prognosis; Recognition; Treatment

Indexed keywords

ALKYLATING AGENT; DOXYCYCLINE; IMMUNOGLOBULIN LIGHT CHAIN; LENALIDOMIDE; MELPHALAN; PROTEASOME INHIBITOR; THALIDOMIDE;

AMYLOIDOSIS; AUTOLOGOUS STEM CELL TRANSPLANTATION; DIAGNOSTIC VALUE; HEART AMYLOIDOSIS; HISTOPATHOLOGY; HUMAN; IMMUNOGLOBULIN LIGHT CHAIN AMYLOIDOSIS; NONHUMAN; NUTRITION; PATHOGENESIS; PHASE 1 CLINICAL TRIAL (TOPIC); PHASE 2 CLINICAL TRIAL (TOPIC); PRIORITY JOURNAL; PROGNOSIS; RANDOMIZED CONTROLLED TRIAL (TOPIC); REVIEW; SCREENING; STEM CELL MOBILIZATION; TEA; CHEMISTRY; METABOLISM; MORTALITY;

AMYLOIDOSIS; HUMANS; IMMUNOGLOBULIN LIGHT CHAINS; PROGNOSIS;

EID: 84955601549     PISSN: 00015792     EISSN: 14219662     Source Type: Journal    
DOI: 10.1159/000443200     Document Type: Review

References (169)

1. Kyle RA. Amyloidosis: A brief history. Amyloid 2011; 18(suppl 1): 6-7
2. Kyle RA. Amyloidosis: A convoluted story. Br J Haematol 2001; 114; 529-538
3. Sipe JD, Benson MD, Buxbaum JN, Ikeda S, Merlini G, Saraiva MJM, Westermark P. Nomenclature 2014: Amyloid fibril proteins and clinical classification of the amyloidosis. Amyloid 2014; 21: 221-224
4. Merlini G, Seldin DC, Gertz M.A. Amyloidosis: pathogenesis and new therapeutic options. J Clin Oncol 2011; 29: 1924-1933
5. Kyle RA, Linos A, Beard CM, Linke RP, Gertz MA, O'Fallon WM, Kurland LT. Incidence and natural history of primary systemic amyloidosis in Olmsted County, Minnesota, 1950 through 1989. Blood 1992; 79: 1817-1822
6. Hemminki K, Li X, Forsti A, Sundquist J, Sundquist K. Incidence and survival in nonhereditary amyloidosis in Sweden. BMC Public Health 2012; 12: 974
7. Pinney JH, Smith CJ, Taube JB, Lachmann HJ, Venner CP, Gibbs SDJ, Dungu J, Banypersad SM, Wechalekar AD, Whelan CJ, Hawkins PN, Gillmore JD. Systemic amyloidosis in England: An epidemiological study. Br J Haematol 2013; 161: 525-532
8. Kourelis TV, Kumar SK, Gertz MA, Lacy MQ, Buadi FK, Hayman SR, Zeldenrust S, Leung N, Kyle RA, Russell S, Dingli D, Lust JA, Lin Y, Kapoor P, Rajkumar SV, McCurdy A, Dispenzieri A. 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; 31: 4319-4324
9. Kourelis TV, Kumar SK, Go RS, Kapoor P, Kyle RA, Buadi FK, Gertz MA, Lacy MQ, Hayman SR, Leung N, Dingli D, Lust JA, Lin Y, Zeldenrust SR, Rajkumar SV, Dispenzieri A. Immunoglobulin light chain amyloidosis is diagnosed late in patients with preexisting plasma cell dyscrasias. Am J Hematol 2014; 89: 1051-1054
10. Weiss BM, Abadie J, Verma P, Howard RS, Kuehl WM. A monoclonal gammopathy precedes multiple myeloma in most patients. Blood 2009; 113: 5418-5422
11. Villar-Pique A, Espargaro A, Ventura S, Sabate R. Screening for amyloid aggregation: insilico, in-vitro and in-vivo detection. Curr Protein Pept Sci 2014; 15: 477-489
12. Ahmed AB, Kajava AV. Breaking the amyloidogenicity code: methods to predict amyloids from amino acid sequence. FEBS Lett 2013; 587: 1089-1095
13. Merlini G, Bellotti V. Molecular mechanisms of amyloidosis. N Engl J Med 2003; 349: 583-596
14. Bellotti V, Nuvolone M, Giorgetti S, Obici L, Palladini G, Russo P, Lavatelli F, Perfetti V, Merlini G. The workings of the amyloid diseases. Ann Med 2007; 39: 200-207
15. Chiti F, Dobson CM. Protein misfolding, functional amyloid, and human disease. Annu Rev Biochem 2006; 75: 333-366
16. McWilliams-Koeppen HP, Foster JS, Hackenbrack N, Ramirez-Alvarado M, Donohoe D, Williams A, Macy S, Wooliver C, Wortham D, Morrell-Falvey J, Foster CM, Kennel SJ, Wall JS. Light chain amyloid fibrils cause metabolic dysfunction in human cardiomyocytes. PloS One 2015; 10: e0137716
17. Shi J, Guan J, Jiang B, Brenner DA, Del Monte F, Ward JE, Connors LH, Sawyer DB, Semigran MJ, Macgillivray TE, Seldin DC, Falk R, Liao R. Amyloidogenic light chains induce cardiomyocyte contractile dysfunction and apoptosis via a non-canonical p38α MAPK pathway. Proc Natl Acad Sci USA 2010; 107: 4188-4193
18. Sikkink LA, Ramirez-Alvarado M. Cytotoxicity of amyloidogenic immunoglobulin light chains in cell culture. Cell Death Dis 2010; 1: e98
19. Zeier M, Perz J, Linke RP, Donini U, Waldherr R, Andrassy K, Ho AD, Goldschmidt H. No regression of renal AL amyloid in monoclonal gammopathy after successful autologous blood stem cell transplantation and significant clinical improvement. Nephrol Dial Transplant 2003; 18: 2644-2647
20. Okuyama H, Yamaya H, Fukusima T, Yokoyama H. A patient with persistent renal AL amyloid deposition after clinical remission by HDM/SCT therapy. Clin Nephrol 2013; 79: 233-236
21. Gertz MA, Kyle RA, Griffing WL, Hunder GG. Jaw claudication in primary systemic amyloidosis. Medicine (Baltimore) 1986; 65: 173-179
22. Matsuda M, Katoh N, Ikeda S. Clinical manifestations at diagnosis in Japanese patients with systemic AL amyloidosis: A retrospective study of 202 cases with a special attention to uncommon symptoms. Intern Med 2014; 53: 403-412
23. Gertz MA, Kyle RA. Myopathy in primary systemic amyloidosis. J Neurol Neurosurg Psychiatry 1996; 60: 655-660
24. Jardinet D, Westhovens R, Peeters J. Sicca syndrome as an initial symptom of amyloidosis. Clin Rheumatol 1998; 17: 546-548
25. Lutz ME, Pittelkow MR. Progressive generalized alopecia due to systemic amyloidosis. J Am Acad Dermatol 2002; 46: 434-436
26. Comenzo RL, Wally J, Kica G, Murray J, Ericsson T, Skinner M, Zhang Y. Clonal immunoglobulin light chain variable region germline gene use in AL amyloidosis: Association with dominant amyloid-related organ involvement and survival after stem cell transplantation. Br J Haematol 1999; 106: 744-751
27. Abraham RS, Geyer SM, Price-Troska TL, Allmer C, Kyle RA, Gertz MA, Fonseca R. Immunoglobulin light chain variable (V) region genes influence clinical presentation and outcome in light chain-associated amyloidosis (AL). Blood 2003; 101: 3801-3808
28. Comenzo RL, Zhang Y, Martinez C, Osman K, Herrera GA. The tropism of organ involvement in primary systemic amyloidosis: contributions of Ig V L germ line gene use and clonal plasma cell burden. Blood 2001; 98: 714-720
29. Bellavia D, Abraham RS, Pellikka PA, Dispenzieri A, Burnett JC, Al-Zahrani GB, Green TD, Manske MK, Gertz MA, Miller FA, Abraham TP. Utility of Doppler myocardial imaging, cardiac biomarkers, and clonal immunoglobulin genes to assess left ventricular performance and stratify risk following peripheral blood stem cell transplantation in patients with systemic light chain amyloidosis (AL). J Am Soc Echocardiogr 2011; 24: 444-454
30. Diomede L, Rognoni P, Lavatelli F, Romeo M, del Favero E, Cantu L, Ghibaudi E, di Fonzo A, Corbelli A, Fiordaliso F, Palladini G, Valentini V, Perfetti V, Salmona M, Merlini G. A Caenorhabditis elegans-based assay recognizes immunoglobulin light chains causing heart amyloidosis. Blood 2014; 123: 3543-3552
31. Dispenzieri A, Gertz MA, Buadi F. What do I need to know about immunoglobulin light chain (AL) amyloidosis?. Blood Rev 2012; 26: 137-154
32. Merlini G, Stone MJ. Dangerous small B-cell clones. Blood 2006; 108; 2520-2530
33. Dispenzieri A, Kyle R, Merlini G, Miguel JS, Ludwig H, Hajek R, Palumbo A, Jagannath S, Blade S, Lonial S, Dimopoulos M, Comenzo R, Einsele H, Barlogie B, Anderson K, Gertz M, Harousseau JL, Attal M, Tosi P, Sonneveld P, Boccadoro M, Morgan G, Richardson P, Sezer O, Mateos MV, Cavo M, Joshua D, Turesson I, Chen W, Shimizu K, Powles R, Rajkumar SV, Durie BGM; International Myeloma Working Group: International Myeloma Working Group guidelines for serum-free light chain analysis in multiple myeloma and related disorders. Leukemia 2009; 23: 215-224
34. Katzmann JA. Screening panels for monoclonal gammopathies: time to change. Clin Biochem Rev Aust Assoc Clin Biochem 2009; 30: 105-111
35. Gertz MA, Lacy MQ, Dispenzieri A, Hayman SR, Kumar SK, Dingli D, Ansell SM, Gastineau DA, Inwards DJ, Johnston PB, Litzow MR, Micallef INM, Porrata LF, Leung N, Hogan WJ, Buadi FK. Autologous stem cell transplant for immunoglobulin light chain amyloidosis: A status report. Leuk Lymphoma 2010; 51: 2181-2187
36. Miyazaki K, Kawai S, Suzuki K. Abdominal subcutaneous fat pad aspiration and bone marrow examination for the diagnosis of AL amyloidosis: the reliability of immunohistochemistry. Int J Hematol 2015; 102: 289-295
37. Shidham VB, Hunt B, Jardeh SS, Barboi AC, Devata S, Hari P. Performing and processing FNA of anterior fat pad for amyloid. J Vis Exp 2010; 44: 1747
38. van Gameren II, Hazenberg BPC, Bijzet J, van Rijswijk MH. Diagnostic accuracy of subcutaneous abdominal fat tissue aspiration for detecting systemic amyloidosis and its utility in clinical practice. Arthritis Rheum 2006; 54: 2015-2021
39. Kyle RA, Gertz M.A. Primary systemic amyloidosis: clinical and laboratory features in 474 cases. Semin Hematol 1995; 32: 45-59
40. Foli A, Palladini G, Caporali R, Verga L, Morbini P, Obici L, Russo P, Sarais G, Donadei S, Montecucco C, Merlini G. The role of minor salivary gland biopsy in the diagnosis of systemic amyloidosis: results of a prospective study in 62 patients. Amyloid 2011; 18(suppl 1): 80-82
41. Vrana JA, Gamez JD, Madden BJ, Theis JD, Bergen HR, Dogan A. Classification of amyloidosis by laser microdissection and mass spectrometry-based proteomic analysis in clinical biopsy specimens. Blood 2009; 114: 4957-4959
42. Leung N, Nasr SH, Sethi S. How I treat amyloidosis: the importance of accurate diagnosis and amyloid typing. Blood 2012; 120: 3206-3213
43. Kyle RA, San-Miguel JF, Mateos M-V, Rajkumar SV. Monoclonal gammopathy of undetermined significance and smoldering multiple myeloma. Hematol Oncol Clin North Am 2014; 28: 775-790
44. Lachmann HJ, Booth DR, Booth SE, Bybee A, Gilbertson JA, Gillmore JD, Pepys MB, Hawkins PN. Misdiagnosis of hereditary amyloidosis as AL (primary) amyloidosis. N Engl J Med 2002; 346: 1786-1791
45. Linke RP. On typing amyloidosis using immunohistochemistry: detailed illustrations, review and a note on mass spectrometry. Prog Histochem Cytochem 2012; 47: 61-132
46. Schonland SO, Hegenbart U, Bochtler T, Mangatter A, Hansberg M, Ho AD, Lohse P, Rocken C. Immunohistochemistry in the classification of systemic forms of amyloidosis: A systematic investigation of 117 patients. Blood 2012; 119: 488-493
47. Arbustini E, Verga L, Concardi M, Palladini G, Obici L, Merlini G. Electron and immunoelectron microscopy of abdominal fat identifies and characterizes amyloid fibrils in suspected cardiac amyloidosis. Amyloid 2002; 9: 108-114
48. Kyle RA, Bayrd ED. Amyloidosis: review of 236 cases. Medicine (Baltimore) 1975; 54: 271-299
49. Bhat A, Selmi C, Naguwa SM, Cheema GS, Gershwin ME. Currents concepts on the immunopathology of amyloidosis. Clin Rev Allergy Immunol 2010; 38: 97-106
50. Westermark P. Localized AL amyloidosis: A suicidal neoplasm?. Ups J Med Sci 2012; 117: 244-250
51. Paccalin M, Hachulla E, Cazalet C, Tricot L, Carreiro M, Rubi M, Grateau G, Roblot P. Localized amyloidosis: A survey of 35 French cases. Amyloid 2005; 12: 239-245
52. Palladini G, Sachchithanantham S, Milani P, Gillmore J, Foli A, Lachmann H, Basset M, Hawkins P, Merlini G, Wechalekar AD. A European collaborative study of cyclophosphamide, bortezomib, and dexamethasone in upfront treatment of systemic AL amyloidosis. Blood 2015; 126: 612-615
53. Skinner M, Sanchorawala V, Seldin DC, Dember LM, Falk RH, Berk JL, Anderson JJ, O'Hara C, Finn KT, Libbey CA, Wiesman J, Quillen K, Swan N, Wright DG. High-dose melphalan and autologous stem-cell transplantation in patients with AL amyloidosis: An 8-year study. Ann Intern Med 2004; 140: 85-93
54. Palladini G, Barassi A, Klersy C, Pacciolla R, Milani P, Sarais G, Perlini S, Albertini R, Russo P, Foli A, Bragotti LZ, Obici L, Moratti R, Melzi d'Eril GV, Merlini G. The combination of high-sensitivity cardiac troponin T (hscTnT) at presentation and changes in N-terminal natriuretic peptide type B (NT-pro- BNP) after chemotherapy best predicts survival in AL amyloidosis. Blood 2010; 116: 3426-3430
55. Palladini G, Campana C, Klersy C, Balduini A, Vadacca G, Perfetti V, Perlini S, Obici L, Ascari E, d? Eril GM, Moratti R, Merlini G. Serum N-terminal pro-brain natriuretic peptide is a sensitive marker of myocardial dysfunction in AL amyloidosis. Circulation 2003; 107: 2440-2445
56. Sharma PP, Payvar S, Litovsky SH. Histomorphometric analysis of intramyocardial vessels in primary and senile amyloidosis: epicardium versus endocardium. Cardiovasc Pathol 2008; 17; 65-71
57. Daubert JP, Gaede J, Cohen HJ. A fatal case of constrictive pericarditis due to a marked, selective pericardial accumulation of amyloid. Am J Med 1993; 94: 335-340
58. Wittich CM, Neben-Wittich MA, Mueller PS, Gertz MA, Edwards WD. Deposition of amyloid proteins in the epicardial coronary arteries of 58 patients with primary systemic amyloidosis. Cardiovasc Pathol 2007; 16: 75-78
59. Gertz MA, Comenzo R, Falk RH, Fermand JP, Hazenberg BP, Hawkins PN, Merlini G, Moreau P, Ronco P, Sanchorawala V, Sezer O, Solomon A, Grateau G. 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: 319-328
60. Suresh R, Grogan M, Maleszewski JJ, Pellikka PA, Hanna M, Dispenzieri A, Pereira NL. Advanced cardiac amyloidosis associated with normal interventricular septal thickness: An uncommon presentation of infiltrative cardiomyopathy. J Am Soc Echocardiogr 2014; 27: 440-447
61. Lee GY, Kim K, Choi J-O, Kim SJ, Kim J-S, Choe YH, Grogan MA, Jeon E-S: Cardiac amyloidosis without increased left ventricular wall thickness. Mayo Clin Proc 2014; 89: 781-789
62. Bellavia D, Pellikka PA, Al-Zahrani GB, Abraham TP, Dispenzieri A, Miyazaki C, Lacy M, Scott CG, Oh JK, Miller FA. Independent predictors of survival in primary systemic (AL) amyloidosis, including cardiac biomarkers and left ventricular strain imaging: An observational cohort study. J Am Soc Echocardiogr 2010; 23: 643-652
63. Hashimura H, Ishibashi-Ueda H, Yonemoto Y, Ohta-Ogo K, Matsuyama T-A, Ikeda Y, Morita Y, Yamada N, Yasui H, Naito H. Late gadolinium enhancement in cardiac amyloidosis: Attributable both to interstitial amyloid deposition and subendocardial fibrosis caused by ischemia. Heart Vessels 2015, Epub ahead of print
64. Syed IS, Glockner JF, Feng D, Araoz PA, Martinez MW, Edwards WD, Gertz MA, Dispenzieri A, Oh JK, Bellavia D, Tajik AJ, Grogan M. Role of cardiac magnetic resonance imaging in the detection of cardiac amyloidosis. JACC Cardiovasc Imaging 2010; 3: 155-164
65. Fontana M, Pica S, Reant P, Abdel-Gadir A, Treibel TA, Banypersad SM, Maestrini V, Barcella W, Rosmini S, Bulluck H, Sayed RH, Patel K, Mahmood S, Bucciarelli-Ducci C, Whelan CJ, Herrey AS, Lachmann HJ, Wechalekar AD, Manisty CH, Schelbert EB, Kellman P, Gillmore JD, Hawkins PN, Moon JC. Prognostic value of late gadolinium enhancement cardiovascular magnetic resonance in cardiac amyloidosis. Circulation 2015; 132: 1570-1579
66. Talamo G, Mir Muhammad A, Pandey MK, Zhu J, Creer MH, Malysz J. Estimation of daily proteinuria in patients with amyloidosis by using the protein-to-creatinine ratio in random urine samples. Rare Tumors 2015; 7: 5686
67. Bergesio F, Ciciani AM, Santostefano M, Brugnano R, Manganaro M, Palladini G, Di Palma AM, Gallo M, Tosi PL, Salvadori M; Immunopathology Group, Italian Society of Nephrology: Renal involvement in systemic amyloidosis - An Italian retrospective study on epidemiological and clinical data at diagnosis. Nephrol Dial Transplant 2007; 22: 1608-1618
68. Eirin A, Irazabal MV, Gertz MA, Dispenzieri A, Lacy MQ, Kumar S, Sethi S, Nasr SH, Cornell LD, Fidler ME, Fervenza FC, Leung N. Clinical features of patients with immunoglobulin light chain amyloidosis (AL) with vascular-limited deposition in the kidney. Nephrol Dial Transplant 2012; 27: 1097-1101
69. Dingli D, Tan TS, Kumar SK, Buadi FK, Dispenzieri A, Hayman SR, Lacy MQ, Gastineau DA, Hogan WJ, Gertz M.A. Stem cell transplantation in patients with autonomic neuropathy due to primary (AL) amyloidosis. Neurology 2010; 74: 913-918
70. Sachchithanantham S, Wechalekar AD. Imaging in systemic amyloidosis. Br Med Bull 2013; 107: 41-56
71. Kyle RA, Gertz MA, Greipp PR, Witzig TE, Lust JA, Lacy MQ, Therneau TM. A trial of three regimens for primary amyloidosis: colchicine alone, melphalan and prednisone, and melphalan, prednisone, and colchicine. N Engl J Med 1997; 336: 1202-1207
72. Castano A, Drachman BM, Judge D, Maurer MS. Natural history and therapy of TTR-cardiac amyloidosis: emerging disease-modifying therapies from organ transplantation to stabilizer and silencer drugs. Heart Fail Rev 2015; 20: 163-178
73. Ng B, Connors LH, Davidoff R, Skinner M, Falk RH. Senile systemic amyloidosis presenting with heart failure: A comparison with light chain-associated amyloidosis. Arch Intern Med 2005; 165: 1425-1429
74. Dispenzieri A, Gertz MA, Kyle RA, Lacy MQ, Burritt MF, Therneau TM, Greipp PR, Witzig TE, Lust JA, Rajkumar SV, Fonseca R, Zeldenrust SR, McGregor CGA, Jaffe AS. Serum cardiac troponins and N-terminal pro-brain natriuretic peptide: A staging system for primary systemic amyloidosis. J Clin Oncol 2004; 22: 3751-3757
75. Kumar S, Dispenzieri A, Lacy MQ, Hayman SR, Buadi FK, Colby C, Laumann K, Zeldenrust SR, Leung N, Dingli D, Greipp PR, Lust JA, Russell SJ, Kyle RA, Rajkumar SV, Gertz M.A. Revised prognostic staging system for light chain amyloidosis incorporating cardiac biomarkers and serum free light chain measurements. J Clin Oncol 2012; 30: 989-995
76. Palladini G, Foli A, Milani P, Russo P, Albertini R, Lavatelli F, Obici L, Perlini S, Moratti R, Merlini G. Best use of cardiac biomarkers in patients with AL amyloidosis and renal failure. Am J Hematol 2012; 87: 465-471
77. Dispenzieri A, Gertz MA, Kumar SK, Lacy MQ, Kyle RA, Saenger AK, Grogan M, Zeldenrust SR, Hayman SR, Buadi F, Greipp PR, Leung N, Russell SR, Dingli D, Lust JA, Rajkumar SV, Jaffe AS. High sensitivity cardiac troponin T in patients with immunoglobulin light chain amyloidosis. Heart Br Card Soc 2014; 100: 383-388
78. Gertz MA, Kyle RA, Greipp PR. Response rates and survival in primary systemic amyloidosis. Blood 1991; 77: 257-262
79. Cordes S, Dispenzieri A, Lacy MQ, Hayman SR, Buadi FK, Dingli D, Kumar SK, Hogan WJ, Gertz M.A. Ten-year survival after autologous stem cell transplantation for immunoglobulin light chain amyloidosis. Cancer 2012; 118: 6105-6109
80. Warsame R, Kumar SK, Gertz MA, Lacy MQ, Buadi FK, Hayman SR, Leung N, Dingli D, Lust JA, Ketterling RP, Lin Y, Russell S, Hwa L, Kapoor P, Go RS, Zeldenrust SR, Kyle RA, Rajkumar SV, Dispenzieri A. Abnormal FISH in patients with immunoglobulin light chain amyloidosis is a risk factor for cardiac involvement and for death. Blood Cancer J 2015; 5: e310
81. Wechalekar AD, Schonland SO, Kastritis E, Gillmore JD, Dimopoulos MA, Lane T, Foli A, Foard D, Milani P, Rannigan L, Hegenbart U, Hawkins PN, Merlini G, Palladini G. A European collaborative study of treatment outcomes in 346 patients with cardiac stage III AL amyloidosis. Blood 2013; 121: 3420-3427
82. Mollee PN, Wechalekar AD, Pereira DL, Franke N, Reece D, Chen C, Stewart AK. Autologous stem cell transplantation in primary systemic amyloidosis: the impact of selection criteria on outcome. Bone Marrow Transplant 2004; 33: 271-277
83. Kumar S, Dispenzieri A, Lacy MQ, Hayman SR, Leung N, Zeldenrust SR, Buadi FK, Kyle RA, Rajkumar SV, Gertz M.A. Serum uric acid: novel prognostic factor in primary systemic amyloidosis. Mayo Clin Proc 2008; 83: 297-303
84. Kumar SK, Gertz MA, Lacy MQ, Dingli D, Hayman SR, Buadi FK, Short-Detweiler K, Zeldenrust SR, Leung N, Greipp PR, Lust JA, Russell SJ, Kyle RA, Rajkumar SV, Dispenzieri A. Recent improvements in survival in primary systemic amyloidosis and the importance of an early mortality risk score. Mayo Clin Proc 2011; 86: 12-18
85. Caccialanza R, Palladini G, Klersy C, Cereda E, Bonardi C, Cameletti B, Quarleri L, Montagna E, Foli A, Milani P, Lavatelli F, Marena C, Merlini G. 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; 38: 891-894
86. Kristen AV, Rosenberg M, Lindenmaier D, Merkle C, Steen H, Andre F, Schonland SO, Schnabel PA, Schuster T, Rocken C, Giannitsis E, Katus HA, Frey N. Osteopontin: A novel predictor of survival in patients with systemic light-chain amyloidosis. Amyloid 2014; 21: 202-210
87. Dispenzieri A, Gertz MA, Saenger A, Kumar SK, Lacy MQ, Buadi FK, Dingli D, Leung N, Zeldenrust S, Hayman SR, Kapoor P, Grogan M, Hwa L, Russell SJ, Go RS, Rajkumar SV, Kyle RA, Jaffe A. Soluble suppression of tumorigenicity 2 (sST2), but not galactin-3, adds to prognostication in patients with systemic AL amyloidosis independent of NTproBNP and troponin T. Am J Hematol 2015; 90: 524-528
88. Kristen AV, Rinn J, Hegenbart U, Lindenmaier D, Merkle C, Rocken C, Hardt S, Giannitsis E, Katus HA. Improvement of risk assessment in systemic light-chain amyloidosis using human placental growth factor. Clin Res Cardiol 2015; 104: 250-257
89. Palladini G, Dispenzieri A, Gertz MA, Kumar S, Wechalekar A, Hawkins PN, Schonland S, Hegenbart U, Comenzo R, Kastritis E, Dimopoulos MA, Jaccard A, Klersy C, Merlini G. 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: 4541-4549
90. Dispenzieri A, Lacy MQ, Katzmann JA, Rajkumar SV, Abraham RS, Hayman SR, Kumar SK, Clark R, Kyle RA, Litzow MR, Inwards DJ, Ansell SM, Micallef IM, Porrata LF, Elliott MA, Johnston PB, Greipp PR, Witzig TE, Zeldenrust SR, Russell SJ, Gastineau D, Gertz M.A. Absolute values of immunoglobulin free light chains are prognostic in patients with primary systemic amyloidosis undergoing peripheral blood stem cell transplantation. Blood 2006; 107: 3378-3383
91. Palladini G, Hegenbart U, Milani P, Kimmich C, Foli A, Ho AD, Vidus Rosin M, Albertini R, Moratti R, Merlini G, Schonland S. A staging system for renal outcome and early markers of renal response to chemotherapy in AL amyloidosis. Blood 2014; 124: 2325-2332
92. Dispenzieri A, Buadi F, Kumar SK, Reeder CB, Sher T, Lacy MQ, Kyle RA, Mikhael JR, Roy V, Leung N, Grogan M, Kapoor P, Lust JA, Dingli D, Go RS, Hwa YL, Hayman SR, Fonseca R, Ailawadhi S, Bergsagel PL, Chanan-Khan A, Rajkumar SV, Russell SJ, Stewart K, Zeldenrust SR, Gertz M.A. Treatment of immunoglobulin light chain amyloidosis: Mayo Stratification of Myeloma and Risk-Adapted Therapy (mSMART) Consensus Statement. Mayo Clin Proc 2015; 90: 1054-1081
93. Kaufman GP, Dispenzieri A, Gertz MA, Lacy MQ, Buadi FK, Hayman SR, Leung N, Dingli D, Lust JA, Lin Y, Kapoor P, Go RS, Zeldenrust SR, Kyle RA, Rajkumar SV, Kumar SK. Kinetics of organ response and survival following normalization of the serum free light chain ratio in AL amyloidosis. Am J Hematol 2015; 90: 181-186
94. Cibeira MT, Sanchorawala V, Seldin DC, Quillen K, Berk JL, Dember LM, Segal A, Ruberg F, Meier-Ewert H, Andrea NT, Sloan JM, Finn KT, Doros G, Blade J, Skinner M. Outcome of AL amyloidosis after high-dose melphalan and autologous stem cell transplantation: long-term results in a series of 421 patients. Blood 2011; 118: 4346-4352
95. Gertz MA, Lacy MQ, Dispenzieri A, Kumar SK, Buadi FK, Dingli D, Leung N, Hogan WJ, Hayman SR. Trends in day 100 and 2-year survival after auto-SCT for AL amyloidosis: outcomes before and after 2006. Bone Marrow Transplant 2011; 46: 970-975
96. Gertz MA, Lacy MQ, Dispenzieri A, Kumar SK, Dingli D, Leung N, Hogan WJ, Buadi FK, Hayman SR. Refinement in patient selection to reduce treatment-related mortality from autologous stem cell transplantation in amyloidosis. Bone Marrow Transplant 2013; 48: 557-561
97. Jantunen E, Itala M, Lehtinen T, Kuittinen O, Koivunen E, Leppa S, Juvonen E, Koistinen P, Wiklund T, Nousiainen T, Remes K, Volin L. Early treatment-related mortality in adult autologous stem cell transplant recipients: A nation-wide survey of 1482 transplanted patients. Eur J Haematol 2006; 76: 245-250
98. D'Souza A, Dispenzieri A, Wirk B, Zhang M-J, Huang J, Gertz MA, Kyle RA, Kumar S, Comenzo RL, Peter Gale R, Lazarus HM, Savani BN, Cornell RF, Weiss BM, Vogl DT, Freytes CO, Scott EC, Landau HJ, Moreb JS, Costa LJ, Ramanathan M, Callander NS, Kamble RT, Olsson RF, Ganguly S, Nishihori T, Kindwall-Keller TL, Wood WA, Mark TM, Hari P. 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; 33: 3741-3749
99. Hegenbart U, Iacobelli S, Hoek J, Rovira M, van Imhoff G, Bandini G, Mellqvist U-H, Leblond V, Carlson K, Zver S, Blaise D, Alessandrino EP, Garderet L, Kroger N. Center experience and calendar year of transplantation strongly influence short term survival after autologous peripheral blood transplantation in 1, 315 patients with light chain amyloidosis: An EBMT analysis. Blood 2013; 122: 417-417
100. Comenzo RL, Vosburgh E, Simms RW, Bergethon P, Sarnacki D, Finn K, Dubrey S, Faller DV, Wright DG, Falk RH, Skinner M. Dose-intensive melphalan with blood stem cell support for the treatment of AL amyloidosis: one-year follow-up in five patients. Blood 1996; 88: 2801-2806
101. Dispenzieri A, Seenithamby K, Lacy MQ, Kumar SK, Buadi FK, Hayman SR, Dingli D, Litzow MR, Gastineau DA, Inwards DJ, Micallef IN, Ansell SM, Johnston PB, Porrata LF, Patnaik MM, Hogan WJ, Gertz M.A. Patients with immunoglobulin light chain amyloidosis undergoing autologous stem cell transplantation have superior outcomes compared with patients with multiple myeloma: A retrospective review from a tertiary referral center. Bone Marrow Transplant 2013; 48: 1302-1307
102. Jaccard A, Moreau P, Leblond V, Leleu X, Benboubker L, Hermine O, Recher C, Asli B, Lioure B, Royer B, Jardin F, Bridoux F, Grosbois B, Jaubert J, Piette J-C, Ronco P, Quet F, Cogne M, J-P, Fermand; Myelome Autogreffe (MAG) and Intergroupe Francophone du Myelome (IFM): High-dose melphalan versus melphalan plus dexamethasone for AL amyloidosis. N Engl J Med 2007; 357: 1083-1093
103. 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; 29: e79-e80
104. Dhakal B, Strouse C, D'Souza A, Arce-Lara C, Esselman J, Eastwood D, Pasquini M, Saber W, Drobyski W, Rizzo JD, Hari PN, Hamadani M. Plerixafor and abbreviatedcourse granulocyte colony-stimulating factor for mobilizing hematopoietic progenitor cells in light chain amyloidosis. Biol Blood Marrow Transplant 2014; 20: 1926-1931
105. Kaul E, Shah G, Chaulagain C, Comenzo RL. Plerixafor and G-CSF for autologous stem cell mobilization in AL amyloidosis. Bone Marrow Transplant 2014; 49: 1233
106. Gertz MA, Lacy MQ, Dispenzieri A, Ansell SM, Elliott MA, Gastineau DA, Inwards DJ, Micallef INM, Porrata LF, Tefferi A, Litzow MR. Risk-adjusted manipulation of melphalan dose before stem cell transplantation in patients with amyloidosis is associated with a lower response rate. Bone Marrow Transplant 2004; 34: 1025-1031
107. Sanchorawala V, Wright DG, Seldin DC, Falk RH, Finn KT, Dember LM, Berk JL, Quillen K, Anderson JJ, Comenzo RL, Skinner M. High-dose intravenous melphalan and autologous stem cell transplantation as initial therapy or following two cycles of oral chemotherapy for the treatment of AL amyloidosis: results of a prospective randomized trial. Bone Marrow Transplant 2004; 33: 381-388
108. Huang X, Wang Q, Chen W, Zeng C, Chen Z, Gong D, Zhang H, Liu Z. Induction therapy with bortezomib and dexamethasone followed by autologous stem cell transplantation versus autologous stem cell transplantation alone in the treatment of renal AL amyloidosis: A randomized controlled trial. BMC Med 2014; 12: 2
109. Landau H, Hassoun H, Rosenzweig MA, Maurer M, Liu J, Flombaum C, Bello C, Hoover E, Riedel E, Giralt S, Comenzo RL. Bortezomib and dexamethasone consolidation following risk-adapted melphalan and stem cell transplantation for patients with newly diagnosed light-chain amyloidosis. Leukemia 2013; 27: 823-828
110. Kastritis E, Roussou M, Gavriatopoulou M, Migkou M, Kalapanida D, Pamboucas C, Kaldara E, Ntalianis A, Psimenou E, Toumanidis ST, Tasidou A, Terpos E, Dimopoulos M.A. Long-term outcomes of primary systemic light chain (AL) amyloidosis in patients treated upfront with bortezomib or lenalidomide and the importance of risk adapted strategies. Am J Hematol 2015; 90: E60-E65
111. Cibeira MT, Oriol A, Lahuerta JJ, Mateos M-V, de la Rubia J, Hernandez MT, Granell M, Fernandez de Larrea C, San Miguel JF, Blade J; PETHEMA cooperative study group: A phase II trial of lenalidomide, dexamethasone and cyclophosphamide for newly diagnosed patients with systemic immunoglobulin light chain amyloidosis. Br J Haematol 2015; 170: 804-813
112. Dhodapkar MV, Hussein MA, Rasmussen E, Solomon A, Larson RA, Crowley JJ, Barlogie B; United States Intergroup Trial Southwest Oncology Group: Clinical efficacy of high-dose dexamethasone with maintenance dexamethasone/alpha interferon in patients with primary systemic amyloidosis: results of United States Intergroup Trial Southwest Oncology Group (SWOG) S9628. Blood 2004; 104: 3520-3526
113. Skinner M, Anderson J, Simms R, Falk R, Wang M, Libbey C, Jones LA, Cohen AS. Treatment of 100 patients with primary amyloidosis: A randomized trial of melphalan, prednisone, and colchicine versus colchicine only. Am J Med 1996; 100: 290-298
114. Palladini G, Perfetti V, Obici L, Caccialanza R, Semino A, Adami F, Cavallero G, Rustichelli R, Virga G, Merlini G. Association of melphalan and high-dose dexamethasone is effective and well tolerated in patients with AL (primary) amyloidosis who are ineligible for stem cell transplantation. Blood 2004; 103: 2936-2938
115. Palladini G, Russo P, Nuvolone M, Lavatelli F, Perfetti V, Obici L, Merlini G. Treatment with oral melphalan plus dexamethasone produces long-term remissions in AL amyloidosis. Blood 2007; 110: 787-788
116. Sanchorawala V, Seldin DC, Berk JL, Sloan JM, Doros G, Skinner M. Oral cyclic melphalan and dexamethasone for patients with AL amyloidosis. Clin Lymphoma Myeloma Leuk 2010; 10: 469-472
117. Dietrich S, Schonland SO, Benner A, Bochtler T, Kristen AV, Beimler J, Hund E, Zorn M, Goldschmidt H, Ho AD, Hegenbart U. Treatment with intravenous melphalan and dexamethasone is not able to overcome the poor prognosis of patients with newly diagnosed systemic light chain amyloidosis and severe cardiac involvement. Blood 2010; 116: 522-528
118. Lebovic D, Hoffman J, Levine BM, Hassoun H, Landau H, Goldsmith Y, Maurer MS, Steingart RM, Cohen AD, Comenzo RL. Predictors of survival in patients with systemic light-chain amyloidosis and cardiac involvement initially ineligible for stem cell transplantation and treated with oral melphalan and dexamethasone. Br J Haematol 2008; 143: 369-373
119. Reece DE, Hegenbart U, Sanchorawala V, Merlini G, Palladini G, Blade J, Fermand J-P, Hassoun H, Heffner L, Vescio RA, Liu K, Enny C, Esseltine D-L, van de Velde H, Cakana A, Comenzo RL. Efficacy and safety of once-weekly and twice-weekly bortezomib in patients with relapsed systemic AL amyloidosis: results of a phase 1/2 study. Blood 2011; 118: 865-873
120. Dubrey SW, Reece DE, Sanchorawala V, Hegenbart U, Merlini G, Palladini G, Fermand J-P, Vescio RA, Blade J, Heffner LT, Hassoun H, Liu X, Enny C, Ramaswami P, Elsayed Y, van de Velde H, Mortimer S, Cakana A, Comenzo RL, Velcade RL; Can2007 Study Group: Bortezomib in a phase 1 trial for patients with relapsed AL amyloidosis: cardiac responses and overall effects. QJM 2011; 104: 957-970
121. Mikhael JR, Schuster SR, Jimenez-Zepeda VH, Bello N, Spong J, Reeder CB, Stewart AK, Bergsagel PL, Fonseca R. Cyclophosphamide- bortezomib-dexamethasone (CyBorD) produces rapid and complete hematologic response in patients with AL amyloidosis. Blood 2012; 119: 4391-4394
122. Kastritis E, Leleu X, Arnulf B, Zamagni E, Cibeira MT, Kwok F, Mollee P, Hajek R, Moreau P, Jaccard A, Schonland S, Filshie R, Nicolas-Virelizier E, Augustson B, Mateos M-V, Wechalekar A, Hachulla E, Milani P, Dimopoulos MA, Fermand J-P, Foli A, Gavriatopoulou M, Palumbo A, Sonneveld P, Johnsen HE, Merlini G, Palladini G. A randomized phase III trial of melphalan and dexamethasone (MDex) versus bortezomib, melphalan and dexamethasone (BMDex) for untreated patients with AL amyloidosis. Clin Lymphoma Myeloma Leuk 2015; 15: e59-e60
123. Seldin DC, Choufani EB, Dember LM, Wiesman JF, Berk JL, Falk RH, O'Hara C, Fennessey S, Finn KT, Wright DG, Skinner M, Sanchorawala V. Tolerability and efficacy of thalidomide for the treatment of patients with light chain-associated (AL) amyloidosis. Clin Lymphoma 2003; 3: 241-246
124. Dispenzieri A, Lacy MQ, Rajkumar SV, Geyer SM, Witzig TE, Fonseca R, Lust JA, Greipp PR, Kyle RA, Gertz M.A. Poor tolerance to high doses of thalidomide in patients with primary systemic amyloidosis. Amyloid 2003; 10: 257-261
125. Palladini G, Perfetti V, Perlini S, Obici L, Lavatelli F, Caccialanza R, Invernizzi R, Comotti B, Merlini G. The combination of thalidomide and intermediate-dose dexamethasone is an effective but toxic treatment for patients with primary amyloidosis (AL). Blood 2005; 105: 2949-2951
126. Wechalekar AD, Goodman HJB, Lachmann HJ, Offer M, Hawkins PN, Gillmore JD. Safety and efficacy of risk-adapted cyclophosphamide, thalidomide, and dexamethasone in systemic AL amyloidosis. Blood 2007; 109: 457-464
127. Venner CP, Gillmore JD, Sachchithanantham S, Mahmood S, Lane T, Foard D, Rannigan L, Gibbs SDJ, Pinney JH, Whelan CJ, Lachmann HJ, Hawkins PN, Wechalekar AD. A matched comparison of cyclophosphamide, bortezomib and dexamethasone (CVD) versus risk-adapted cyclophosphamide, thalidomide and dexamethasone (CTD) in AL amyloidosis. Leukemia 2014; 28: 2304-2310
128. Dispenzieri A, Lacy MQ, Zeldenrust SR, Hayman SR, Kumar SK, Geyer SM, Lust JA, Allred JB, Witzig TE, Rajkumar SV, Greipp PR, Russell SJ, Kabat B, Gertz M.A. The activity of lenalidomide with or without dexamethasone in patients with primary systemic amyloidosis. Blood 2007; 109: 465-470
129. Sanchorawala V, Wright DG, Rosenzweig M, Finn KT, Fennessey S, Zeldis JB, Skinner M, Seldin DC. Lenalidomide and dexamethasone in the treatment of AL amyloidosis: results of a phase 2 trial. Blood 2007; 109: 492-496
130. Moreau P, Jaccard A, Benboubker L, Royer B, Leleu X, Bridoux F, Salles G, Leblond V, Roussel M, Alakl M, Hermine O, Planche L, Harousseau J-L, Fermand J-P: Lenalidomide in combination with melphalan and dexamethasone in patients with newly diagnosed AL amyloidosis: A multicenter phase 1/2 dose-escalation study. Blood 2010; 116: 4777-4782
131. Sanchorawala V, Patel JM, Sloan JM, Shelton AC, Zeldis JB, Seldin DC. Melphalan, lenalidomide and dexamethasone for the treatment of immunoglobulin light chain amyloidosis: results of a phase II trial. Haematologica 2013; 98: 789-792
132. Dinner S, Witteles W, Afghahi A, Witteles R, Arai S, Lafayette R, Schrier SL, Liedtke M. Lenalidomide, melphalan and dexamethasone in a population of patients with immunoglobulin light chain amyloidosis with high rates of advanced cardiac involvement. Haematologica 2013; 98: 1593-1599
133. Kastritis E, Terpos E, Roussou M, Gavriatopoulou M, Pamboukas C, Boletis I, Marinaki S, Apostolou T, Nikitas N, Gkortzolidis G, Michalis E, Delimpasi S, Dimopoulos M.A. A phase 1/2 study of lenalidomide with lowdose oral cyclophosphamide and low-dose dexamethasone (RdC) in AL amyloidosis. Blood 2012; 119: 5384-5390
134. Kumar SK, Hayman SR, Buadi FK, Roy V, Lacy MQ, Gertz MA, Allred J, Laumann KM, Bergsagel LP, Dingli D, Mikhael JR, Reeder CB, Stewart AK, Zeldenrust SR, Greipp PR, Lust JA, Fonseca R, Russell SJ, Rajkumar SV, Dispenzieri A. Lenalidomide, cyclophosphamide, and dexamethasone (CRd) for light-chain amyloidosis: longterm results from a phase 2 trial. Blood 2012; 119: 4860-4867
135. Palladini G, Russo P, Milani P, Foli A, Lavatelli F, Nuvolone M, Perlini S, Merlini G. A phase II trial of cyclophosphamide, lenalidomide and dexamethasone in previously treated patients with AL amyloidosis. Haematologica 2013; 98: 433-436
136. Specter R, Sanchorawala V, Seldin DC, Shelton A, Fennessey S, Finn KT, Zeldis JB, Dember LM. Kidney dysfunction during lenalidomide treatment for AL amyloidosis. Nephrol Dial Transplant 2011; 26: 881-886
137. Dispenzieri A, Dingli D, Kumar SK, Rajkumar SV, Lacy MQ, Hayman S, Buadi F, Zeldenrust S, Leung N, Detweiler-Short K, Lust JA, Russell SJ, Kyle RA, Gertz M.A. 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; 85: 757-759
138. Tapan U, Seldin DC, Finn KT, Fennessey S, Shelton A, Zeldis JB, Sanchorawala V. Increases in B-type natriuretic peptide (BNP) during treatment with lenalidomide in AL amyloidosis. Blood 2010; 116: 5071-5072
139. Dispenzieri A, Buadi F, Laumann K, LaPlant B, Hayman SR, Kumar SK, Dingli D, Zeldenrust SR, Mikhael JR, Hall R, Rajkumar SV, Reeder C, Fonseca R, Bergsagel PL, Stewart AK, Roy V, Witzig TE, Lust JA, Russell SJ, Gertz MA, Lacy MQ. Activity of pomalidomide in patients with immunoglobulin light-chain amyloidosis. Blood 2012; 119: 5397-5404
140. Dubrey SW, Bilazarian S, LaValley M, Reisinger J, Skinner M, Falk RH. Signal-averaged electrocardiography in patients with AL (primary) amyloidosis. Am Heart J 1997; 134: 994-1001
141. Grogan M, Dispenzieri A. Natural history and therapy of AL cardiac amyloidosis. Heart Fail Rev 2015; 20: 155-162
142. Kristen AV, Dengler TJ, Hegenbart U, Schonland SO, Goldschmidt H, Sack F-U, Voss F, Becker R, Katus HA, Bauer A. Prophylactic implantation of cardioverter-defibrillator in patients with severe cardiac amyloidosis and high risk for sudden cardiac death. Heart Rhythm 2008; 5: 235-240
143. Varr BC, Zarafshar S, Coakley T, Liedtke M, Lafayette RA, Arai S, Schrier SL, Witteles RM. Implantable cardioverter-defibrillator placement in patients with cardiac amyloidosis. Heart Rhythm 2014; 11: 158-162
144. Lin G, Dispenzieri A, Kyle R, Grogan M, Brady PA. Implantable cardioverter defibrillators in patients with cardiac amyloidosis. J Cardiovasc Electrophysiol 2013; 24: 793-798
145. Falk RH. Cardiac amyloidosis: A treatable disease, often overlooked. Circulation 2011; 124: 1079-1085
146. Gertz MA, Falk RH, Skinner M, Cohen AS, Kyle RA. Worsening of congestive heart failure in amyloid heart disease treated by calcium channel-blocking agents. Am J Cardiol 1985; 55: 1645
147. Rubinow A, Skinner M, Cohen AS. Digoxin sensitivity in amyloid cardiomyopathy. Circulation 1981; 63: 1285-1288
148. Falk RH, Dubrey SW. Amyloid heart disease. Prog Cardiovasc Dis 2010; 52: 347-361
149. Grupper A, Park SJ, Pereira NL, Schettle SD, Gerber Y, Topilsky Y, Edwards BS, Daly RC, Stulak JM, Joyce LD, Kushwaha SS. Role of ventricular assist therapy for patients with heart failure and restrictive physiology: improving outcomes for a lethal disease. J Heart Lung Transplant 2015; 34: 1042-1049
150. US Food and Drug Administration: Serious Adverse Events with Implantable Left Ventricular Assist Devices: FDA Safety Communication. August 5, 2015
151. Freeman R. Clinical practice: neurogenic orthostatic hypotension. N Engl J Med 2008; 358: 615-624
152. Caccialanza R, Palladini G, Klersy C, Cena H, Vagia C, Cameletti B, Russo P, Lavatelli F, Merlini G. Nutritional status of outpatients with systemic immunoglobulin lightchain amyloidosis 1. Am J Clin Nutr 2006; 83: 350-354
153. Caccialanza R, Palladini G, Klersy C, Cereda E, Bonardi C, Cameletti B, Montagna E, Russo P, Foli A, Milani P, Lavatelli F, Merlini G. Nutritional status independently affects quality of life of patients with systemic immunoglobulin light-chain (AL) amyloidosis. Ann Hematol 2012; 91: 399-406
154. Sattianayagam PT, Lane T, Fox Z, Petrie A, Gibbs SDJ, Pinney JH, Risom SS, Rowczenio DM, Wechalekar AD, Lachmann HJ, Gilbertson JA, Hawkins PN, Gillmore JD. A prospective study of nutritional status in immunoglobulin light chain amyloidosis. Haematologica 2013; 98: 136-140
155. Bodin K, Ellmerich S, Kahan MC, Tennent GA, Loesch A, Gilbertson JA, Hutchinson WL, Mangione PP, Gallimore JR, Millar DJ, Minogue S, Dhillon AP, Taylor GW, Bradwell AR, Petrie A, Gillmore JD, Bellotti V, Botto M, Hawkins PN, Pepys MB. Antibodies to human serum amyloid P component eliminate visceral amyloid deposits. Nature 2010; 468: 93-97
156. Richards DB, Cookson LM, Berges AC, Barton SV, Lane T, Ritter JM, Fontana M, Moon JC, Pinzani M, Gillmore JD, Hawkins PN, Pepys MB. Therapeutic clearance of amyloid by antibodies to serum amyloid P component. N Engl J Med 2015; 373: 1106-1114
157. Gertz MA, Landau HJ, Comenzo R, Seldin DC, Weiss BM, Zonder JA, Walling J, Kinney G, Koller M, Liedtke M. Cardiac and renal biomarker responses in a phase 1/2 study of NEOD001 in patients with AL amyloidosis and persistent organ dysfunction (abstract). J Clin Oncol 2015; 33(suppl): 8514
158. ClinicalTrials.gov: The VITAL Amyloidosis Study, a global phase 3, efficacy and safety study of NEOD001 in patients with AL amyloidosis. https://clinicaltrials.gov/ct2/show/NCT02312206
159. Ward JE, Ren R, Toraldo G, Soohoo P, Guan J, O'Hara C, Jasuja R, Trinkaus-Randall V, Liao R, Connors LH, Seldin DC. Doxycycline reduces fibril formation in a transgenic mouse model of AL amyloidosis. Blood 2011; 118: 6610-6617
160. Wechalekar A, Whelan C, Sachchithanantham S, Fontana M, Mahmood S, Foard D, Lane T, Lachmann HJ, Gillmore JD, Hawkins PN. A matched case control study of doxycycline added to chemotherapy for reducing early mortality in patients with advanced cardiac AL amyloidosis from the Alchemy Study cohort. Blood 2014; 124: 3485-3485
161. ClinicalTrials.gov: Oral doxycycline administered as an adjunct to plasma cell directed therapy in light chain amyloidosis. https://clinicaltrials.gov/ct2/show/NCT02207556?. term=NCT02207556&rank=1
162. ClinicalTrials.gov: Safety and effect of doxycycline in patients with amyloidosis. https://clinicaltrials.gov/ct2/show/NCT01677286?. term=NCT01677286&rank=1
163. Hunstein W. Epigallocathechin-3-gallate in AL amyloidosis: A new therapeutic option?. Blood 2007; 110: 2216
164. Mereles D, Buss SJ, Hardt SE, Hunstein W, Katus HA. Effects of the main green tea polyphenol epigallocatechin-3-gallate on cardiac involvement in patients with AL amyloidosis. Clin Res Cardiol 2010; 99: 483-490
165. Stefani M, Rigacci S. Beneficial properties of natural phenols: highlight on protection against pathological conditions associated with amyloid aggregation. Biofactors 2014; 40: 482-493
166. aus dem Siepen F, Buss SJ, Andre F, Seitz S, Giannitsis E, Steen H, Katus HA, Kristen AV. Extracellular remodeling in patients with wild-type amyloidosis consuming epigallocatechin-3-gallate: preliminary results of T1 mapping by cardiac magnetic resonance imaging in a small single center study. Clin Res Cardiol 2015; 104: 640-647
167. Kristen AV, Lehrke S, Buss S, Mereles D, Steen H, Ehlermann P, Hardt S, Giannitsis E, Schreiner R, Haberkorn U, Schnabel PA, Linke RP, Rocken C, Wanker EE, Dengler TJ, Altland K, Katus HA. Green tea halts progression of cardiac transthyretin amyloidosis: An observational report. Clin Res Cardiol 2012; 101: 805-813
168. ClinicalTrials.gov: A trial for the treatment of cardiac AL-amyloidosis with the green tea compound epigallocatechin-3-gallate (TAME-AL). https://clinicaltrials.gov/ct2/show/NCT02015312?term=NCT02015312 &rank=1
169. Golden EB, Lam PY, Kardosh A, Gaffney KJ, Cadenas E, Louie SG, Petasis NA, Chen TC, Schonthal AH. Green tea polyphenols block the anticancer effects of bortezomib and other boronic acid-based proteasome inhibitors. Blood 2009; 113: 5927-5937