Chapter 1 Amyloidosis

Advances in Clinical Chemistry

Volumn 47, Issue , 2009, Pages 1-44

  Sideras, Kostandinos ^a   Gertz, Morie A ^a  

^a Mayo Clinic College of Medicine   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

AMYLOIDOSIS; CLASSIFICATION; HUMAN; PATHOPHYSIOLOGY; PROGNOSIS; REVIEW;

AMYLOIDOSIS; HUMANS; PROGNOSIS;

EID: 67651244309     PISSN: 00652423     EISSN: None     Source Type: Book Series    
DOI: 10.1016/S0065-2423(09)47001-X     Document Type: Review

References (154)

1. Sipe J.D. Amyloid Proteins. The Beta Sheet Conformation and Disease (2005), Wiley-VCH, Weinheim
2. Sipe J.D., and Cohen A.S. Review: history of the amyloid fibril. J. Struct. Biol. 130 2-3 (2000) 88-98
3. Virchow R.L.K. Cellular Pathology (1860), DeWitt RM, New York [Translated from the 2nd ed of the original by F Chance ed]
4. Friedreich N., and Kekule A. zur amyloidfrage. Virchows Arch. Pathol. Anat. 16 (1859) 50-65
5. Cohen A.S., and Calkins E. Electron microscopic observations on a fibrous component in amyloid of diverse origins. Nature 183 (1959) 1202-1203
6. Eanes E.D., and Glenner G.G. X-ray diffraction studies on amyloid filaments. J. Histochem. Cytochem. 16 11 (1968) 673-677
7. Sunde M., Serpell L.C., Bartlam M., Fraser P.E., Pepys M.B., and Blake C.C. Common core structure of amyloid fibrils by synchrotron X-ray diffraction. J. Mol. Biol. 273 3 (1997) 729-739
8. Blake C., and Serpell L. Synchrotron X-ray studies suggest that the core of the transthyretin amyloid fibril is a continuous beta-sheet helix. Structure 4 8 (1996) 989-998
9. Nelson R., Sawaya M.R., Balbirnie M., Madsen A.O., Riekel C., Grothe R., et al. Structure of the cross-beta spine of amyloid-like fibrils. Nature 435 7043 (2005) 773-778
10. Westermark P., Benson M.D., Buxbaum J.N., Cohen A.S., Frangione B., Ikeda S., et al. Amyloid: toward terminology clarification. Report from the Nomenclature Committee of the International Society of Amyloidosis. Amyloid 12 1 (2005) 1-4
11. Serpell L.C., Sunde M., Benson M.D., Tennent G.A., Pepys M.B., and Fraser P.E. The protofilament substructure of amyloid fibrils. J. Mol. Biol. 300 5 (2000) 1033-1039
12. Nelson R., and Eisenberg D. Recent atomic models of amyloid fibril structure. Curr. Opin. Struct. Biol. 16 2 (2006) 260-265
13. Khurana R., Gillespie J.R., Talapatra A., Minert L.J., Ionescu-Zanetti C., Millett I., et al. Partially folded intermediates as critical precursors of light chain amyloid fibrils and amorphous aggregates. Biochemistry 40 12 (2001) 3525-3535
14. Khurana R., Souillac P.O., Coats A.C., Minert L., Ionescu-Zanetti C., Carter S.A., et al. A model for amyloid fibril formation in immunoglobulin light chains based on comparison of amyloidogenic and benign proteins and specific antibody binding. Amyloid 10 2 (2003) 97-109
15. Qin Z., Hu D., Zhu M., and Fink A.L. Structural characterization of the partially folded intermediates of an immunoglobulin light chain leading to amyloid fibrillation and amorphous aggregation. Biochemistry 46 11 (2007) 3521-3531
16. Eakin C.M., Attenello F.J., Morgan C.J., and Miranker A.D. Oligomeric assembly of native-like precursors precedes amyloid formation by beta-2 microglobulin. Biochemistry 43 24 (2004) 7808-7815
17. Walsh D.M., Lomakin A., Benedek G.B., Condron M.M., and Teplow D.B. Amyloid beta-protein fibrillogenesis. Detection of a protofibrillar intermediate. J. Biol. Chem. 272 35 (1997) 22364-22372
18. Walsh D.M., Hartley D.M., Kusumoto Y., Fezoui Y., Condron M.M., Lomakin A., et al. Amyloid beta-protein fibrillogenesis. Structure and biological activity of protofibrillar intermediates. J. Biol. Chem. 274 36 (1999) 25945-25952
19. Nichols M.R., Moss M.A., Reed D.K., Lin W.L., Mukhopadhyay R., Hoh J.H., et al. Growth of beta-amyloid(1-40) protofibrils by monomer elongation and lateral association. Characterization of distinct products by light scattering and atomic force microscopy. Biochemistry 41 19 (2002) 6115-6127
20. Witte T., Haller L.A., Luttmann E., Kruger J., Fels G., and Huber K. Time resolved structure analysis of growing beta-amyloid fibers. J. Struct. Biol. 159 1 (2007) 71-81
21. Gorbenko G.P., and Kinnunen P.K. The role of lipid-protein interactions in amyloid-type protein fibril formation. Chem. Phys. Lipids 141 1-2 (2006) 72-82
22. Westermark P., Pitkanen P., Benson L., Vahlquist A., Olofsson B.O., and Cornwell III G.G. Serum prealbumin and retinol-binding protein in the prealbumin-related senile and familial forms of systemic amyloidosis. Lab. Invest. 52 3 (1985) 314-318
23. Pepys M.B., Dyck R.F., de Beer F.C., Skinner M., and Cohen A.S. Binding of serum amyloid P-component (SAP) by amyloid fibrils. Clin. Exp. Immunol. 38 2 (1979) 284-293
24. Skinner M., Shirahama T., Cohen A.S., and Deal C.L. The association of amyloid P-component (AP) with the amyloid fibril: an updated method for amyloid fibril protein isolation. Prep. Biochem. 12 5 (1982) 461-476
25. Tennent G.A., Lovat L.B., and Pepys M.B. Serum amyloid P component prevents proteolysis of the amyloid fibrils of Alzheimer disease and systemic amyloidosis. Proc. Natl. Acad. Sci. USA 92 10 (1995) 4299-4303
26. Bottazzi B., Garlanda C., Salvatori G., Jeannin P., Manfredi A., and Mantovani A. Pentraxins as a key component of innate immunity. Curr. Opin. Immunol. 18 1 (2006) 10-15
27. Jager P.L., Hazenberg B.P., Franssen E.J., Limburg P.C., van Rijswijk M.H., and Piers D.A. Kinetic studies with iodine-123-labeled serum amyloid P component in patients with systemic AA and AL amyloidosis and assessment of clinical value. J. Nucl. Med. 39 4 (1998) 699-706
28. 123I-labeled serum amyloid P component scintigraphy in patients with amyloidosis. Am. J. Med. 119 4 (2006) 355 e15 355 e24
29. Magnus J.H., Stenstad T., Husby G., and Kolset S.O. Isolation and partial characterization of heparan sulphate proteoglycans from human hepatic amyloid. Biochem. J. 288 Pt. 1 (1992) 225-231
30. Calamai M., Kumita J.R., Mifsud J., Parrini C., Ramazzotti M., Ramponi G., et al. Nature and significance of the interactions between amyloid fibrils and biological polyelectrolytes. Biochemistry 45 42 (2006) 12806-12815
31. Ginsberg S.D., Galvin J.E., Chiu T.S., Lee V.M., Masliah E., and Trojanowski J.Q. RNA sequestration to pathological lesions of neurodegenerative diseases. Acta Neuropathol. (Berl.) 96 5 (1998) 487-494
32. Benson M.D. Ostertag revisited: the inherited systemic amyloidoses without neuropathy. Amyloid 12 2 (2005) 75-87
33. Comenzo R.L., Zhang Y., Martinez C., Osman K., and Herrera G.A. The tropism of organ involvement in primary systemic amyloidosis: contributions of Ig V(L) germ line gene use and clonal plasma cell burden. Blood 98 3 (2001) 714-720
34. Marx J. Neuroscience. New leads on the 'how' of Alzheimer's. Science 293 5538 (2001) 2192-2194
35. Hartley D.M., Walsh D.M., Ye C.P., Diehl T., Vasquez S., Vassilev P.M., et al. Protofibrillar intermediates of amyloid beta-protein induce acute electrophysiological changes and progressive neurotoxicity in cortical neurons. J. Neurosci. 19 20 (1999) 8876-8884
36. Lambert M.P., Barlow A.K., Chromy B.A., Edwards C., Freed R., Liosatos M., et al. Diffusible, nonfibrillar ligands derived from Abeta1-42 are potent central nervous system neurotoxins. Proc. Natl. Acad. Sci. USA 95 11 (1998) 6448-6453
37. Andersson K., Olofsson A., Nielsen E.H., Svehag S.E., and Lundgren E. Only amyloidogenic intermediates of transthyretin induce apoptosis. Biochem. Biophys. Res. Commun. 294 2 (2002) 309-314
38. Haas M., Meehan S.M., Karrison T.G., and Spargo B.H. Changing etiologies of unexplained adult nephrotic syndrome: a comparison of renal biopsy findings from 1976-1979 and 1995-1997. Am. J. Kidney Dis. 30 5 (1997) 621-631
39. Abraham R.S., Katzmann J.A., Clark R.J., Bradwell A.R., Kyle R.A., and Gertz M.A. Quantitative analysis of serum free light chains. A new marker for the diagnostic evaluation of primary systemic amyloidosis. Am. J. Clin. Pathol. 119 2 (2003) 274-278
40. Linke R.P. Highly sensitive diagnosis of amyloid and various amyloid syndromes using Congo red fluorescence. Virchows Arch. 436 5 (2000) 439-448
41. Cui D., Hoshii Y., Takahashi M., Kawano H., Iwata T., and Ishihara T. An immunohistochemical study of amyloid P component, apolipoprotein E and ubiquitin in human and murine amyloidoses. Pathol. Int. 48 5 (1998) 362-367
42. Lachmann H.J., Booth D.R., Booth S.E., Bybee A., Gilbertson J.A., Gillmore J.D., et al. Misdiagnosis of hereditary amyloidosis as AL (primary) amyloidosis. N. Engl. J. Med. 346 23 (2002) 1786-1791
43. Gangane N., Anshu V.B., Shivkumar, and Sharta S. Cytodiagnosis of hepatic amyloidosis by fine needle aspiration cytology: a case report. Acta Cytol. 50 5 (2006) 574-576
44. Kristen A.V., Dengler T.J., and Katus H.A. Suspected cardiac amyloidosis: endomyocardial biopsy remains the diagnostic gold-standard. Am. J. Hematol. 82 4 (2007) 328
45. Zumrutdal A., Ozelsancak R., Bolat F., Sezerl S., and Ozdemir F.N. Duodenal biopsy may be preferred first for diagnosis of renal amyloidosis in patients with nephrotic syndrome. Clin. Nephrol. 64 3 (2005) 242-243
46. Lechapt-Zalcman E., Authier F.J., Creange A., Voisin M.C., and Gherardi R.K. Labial salivary gland biopsy for diagnosis of amyloid polyneuropathy. Muscle Nerve 22 1 (1999) 105-107
47. Duston M.A., Skinner M., Shirahama T., and Cohen A.S. Diagnosis of amyloidosis by abdominal fat aspiration. Analysis of four years' experience. Am. J. Med. 82 3 (1987) 412-414
48. Gertz M.A., Li C.Y., Shirahama T., and Kyle R.A. Utility of subcutaneous fat aspiration for the diagnosis of systemic amyloidosis (immunoglobulin light chain). Arch. Intern. Med. 148 4 (1988) 929-933
49. Olsen K.E., Sletten K., and Westermark P. The use of subcutaneous fat tissue for amyloid typing by enzyme-linked immunosorbent assay. Am. J. Clin. Pathol. 111 3 (1999) 355-362
50. Guy C.D., and Jones C.K. Abdominal fat pad aspiration biopsy for tissue confirmation of systemic amyloidosis: specificity, positive predictive value, and diagnostic pitfalls. Diagn. Cytopathol. 24 3 (2001) 181-185
51. Pirson Y. From renal amyloid deposits to the identification of the culprit genes. J. Nephrol. 16 3 (2003) 427-430
52. Kaplan B., Martin B.M., Livneh A., Pras M., and Gallo G.R. Biochemical subtyping of amyloid in formalin-fixed tissue samples confirms and supplements immunohistologic data. Am. J. Clin. Pathol. 121 6 (2004) 794-800
53. Kaplan B., Shtrasburg S., and Pras M. Micropurification techniques in the analysis of amyloid proteins. J. Clin. Pathol. 56 2 (2003) 86-90
54. Kebbel A., and Rocken C. Immunohistochemical classification of amyloid in surgical pathology revisited. Am. J. Surg. Pathol. 30 6 (2006) 673-683
55. Linke R.P., Oos R., Wiegel N.M., and Nathrath W.B. Classification of amyloidosis: misdiagnosing by way of incomplete immunohistochemistry and how to prevent it. Acta Histochem. 108 3 (2006) 197-208
56. Kuci H., Ebert M.P., and Rocken C. Anti-lambda-light chain-peptide antibodies are suitable for the immunohistochemical classification of AL amyloid. Histol. Histopathol. 22 4 (2007) 379-387
57. Hazenberg B.P., Bijzet J., Limburg P.C., Skinner M., Hawkins P.N., Butrimiene I., et al. Diagnostic performance of amyloid A protein quantification in fat tissue of patients with clinical AA amyloidosis. Amyloid 14 2 (2007) 133-140
58. Benson M.D., Yazaki M., and Magy N. Laboratory assessment of transthyretin amyloidosis. Clin. Chem. Lab. Med. 40 12 (2002) 1262-1265
59. Murphy C.L., Eulitz M., Hrncic R., Sletten K., Westermark P., Williams T., et al. Chemical typing of amyloid protein contained in formalin-fixed paraffin-embedded biopsy specimens. Am. J. Clin. Pathol. 116 1 (2001) 135-142
60. Murphy C.L., Wang S., Williams T., Weiss D.T., and Solomon A. Characterization of systemic amyloid deposits by mass spectrometry. Methods Enzymol. 412 (2006) 48-62
61. Abraham R.S., Ballman K.V., Dispenzieri A., Grill D.E., Manske M.K., Price-Troska T.L., et al. Functional gene expression analysis of clonal plasma cells identifies a unique molecular profile for light chain amyloidosis. Blood 105 2 (2005) 794-803
62. Arbustini E., Verga L., Concardi M., Palladini G., Obici L., and Merlini G. Electron and immuno-electron microscopy of abdominal fat identifies and characterizes amyloid fibrils in suspected cardiac amyloidosis. Amyloid 9 2 (2002) 108-114
63. Westermark P., Davey E., Lindbom K., and Enqvist S. Subcutaneous fat tissue for diagnosis and studies of systemic amyloidosis. Acta Histochem. 108 3 (2006) 209-213
64. Comenzo R.L., Zhou P., Fleisher M., Clark B., and Teruya-Feldstein J. Seeking confidence in the diagnosis of systemic AL (Ig light-chain) amyloidosis: patients can have both monoclonal gammopathies and hereditary amyloid proteins. Blood 107 9 (2006) 3489-3491
65. Gertz M.A., Kyle R.A., and Thibodeau S.N. Familial amyloidosis: a study of 52 North American-born patients examined during a 30-year period. Mayo Clin. Proc. 67 5 (1992) 428-440
66. Rahman J.E., Helou E.F., Gelzer-Bell R., Thompson R.E., Kuo C., Rodriguez E.R., et al. Noninvasive diagnosis of biopsy-proven cardiac amyloidosis. J. Am. Coll. Cardiol. 43 3 (2004) 410-415
67. Lindqvist P., Olofsson B.O., Backman C., Suhr O., and Waldenstrom A. Pulsed tissue Doppler and strain imaging discloses early signs of infiltrative cardiac disease: a study on patients with familial amyloidotic polyneuropathy. Eur. J. Echocardiogr. 7 1 (2006) 22-30
68. Sallach J.A., and Klein A.L. Tissue Doppler imaging in the evaluation of patients with cardiac amyloidosis. Curr. Opin. Cardiol. 19 5 (2004) 464-471
69. Crean A., and Merchant N. Role of cardiac magnetic resonance imaging in identification of amyloid cardiomyopathy. Indian Heart J. 56 6 (2004) 682-683
70. Kwong R.Y., and Falk R.H. Cardiovascular magnetic resonance in cardiac amyloidosis. Circulation 111 2 (2005) 122-124
71. Maceira A.M., Joshi J., Prasad S.K., Moon J.C., Perugini E., Harding I., et al. Cardiovascular magnetic resonance in cardiac amyloidosis. Circulation 111 2 (2005) 186-193
72. Miyata M., Sato N., Watanabe H., Kumakawa H., Saito A., Funabashi H., et al. Magnetic resonance imaging findings in primary amyloidosis-associated arthropathy. Intern. Med. 39 4 (2000) 313-319
73. Kodama K., Hamada M., Kuwahara T., Nakamura M., Shigematsu Y., Hiwada K., et al. Rest-redistribution thallium-201 myocardial scintigraphic study in cardiac amyloidosis. Int. J. Card. Imaging 15 5 (1999) 371-378
74. Yen T.C., Tzen K.Y., Chen K.S., and Tsai C.J. The value of gallium-67 and thallium-201 whole-body and single-photon emission tomography images in dialysis-related beta 2-microglobulin amyloid. Eur. J. Nucl. Med. 27 1 (2000) 56-61
75. Dezutter N.A., Sciot R.M., de Groot T.J., Bormans G.M., and Verbruggen A.M. In vitro affinity of 99Tcm-labelled N2S2 conjugates of chrysamine G for amyloid deposits of systemic amyloidosis. Nucl. Med. Commun. 22 5 (2001) 553-558
76. Perugini E., Guidalotti P.L., Salvi F., Cooke R.M., Pettinato C., Riva L., et al. Noninvasive etiologic diagnosis of cardiac amyloidosis using 99mtc-3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy. J. Am. Coll. Cardiol. 46 6 (2005) 1076-1084
77. Gillmore J.D., Madhoo S., Pepys M.B., and Hawkins P.N. Renal transplantation for amyloid end-stage renal failure-insights from serial serum amyloid P component scintigraphy. Nucl. Med. Commun. 21 8 (2000) 735-740
78. Hawkins P.N. Serum amyloid P component scintigraphy for diagnosis and monitoring amyloidosis. Curr. Opin. Nephrol. Hypertens 11 6 (2002) 649-655
79. Nordberg A. Amyloid plaque imaging in vivo: current achievement and future prospects. Eur. J. Nucl. Med. Mol. Imaging 35 (2008) 46-50
80. Klunk W.E., Engler H., Nordberg A., Wang Y., Blomqvist G., Holt D.P., et al. Imaging brain amyloid in Alzheimer's disease with Pittsburgh Compound-B. Ann. Neurol. 55 3 (2004) 306-319
81. Engler H., Forsberg A., Almkvist O., Blomquist G., Larsson E., Savitcheva I., et al. Two-year follow-up of amyloid deposition in patients with Alzheimer's disease. Brain 129 Pt. 11 (2006) 2856-2866
82. Perfetti V., Casarini S., Palladini G., Vignarelli M.C., Klersy C., Diegoli M., 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 100 3 (2002) 948-953
83. Gertz M.A., Lacy M.Q., Dispenzieri A., and Hayman S.R. Amyloidosis: diagnosis and management. Clin. Lymphoma Myeloma 6 3 (2005) 208-219
84. Rajkumar S.V., Gertz M.A., and Kyle R.A. Prognosis of patients with primary systemic amyloidosis who present with dominant neuropathy. Am. J. Med. 104 3 (1998) 232-237
85. Comenzo R.L. Primary systemic amyloidosis. Curr. Treat. Options Oncol. 1 1 (2000) 83-89
86. Dispenzieri A., Gertz M.A., Kyle R.A., Lacy M.Q., Burritt M.F., Therneau T.M., 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 104 6 (2004) 1881-1887
87. Dispenzieri A., Gertz M.A., Kyle R.A., Lacy M.Q., Burritt M.F., Therneau T.M., et al. Serum cardiac troponins and N-terminal pro-brain natriuretic peptide: a staging system for primary systemic amyloidosis. J. Clin. Oncol. 22 18 (2004) 3751-3757
88. Dispenzieri A., Lacy M.Q., Katzmann J.A., Rajkumar S.V., Abraham R.S., Hayman S.R., et al. Absolute values of immunoglobulin free light chains are prognostic in patients with primary systemic amyloidosis undergoing peripheral blood stem cell transplantation. Blood 107 8 (2006) 3378-3383
89. McAdam K.P., and Sipe J.D. Murine model for human secondary amyloidosis: genetic variability of the acute-phase serum protein SAA response to endotoxins and casein. J. Exp. Med. 144 4 (1976) 1121-1127
90. Clifton P.M., Mackinnon A.M., and Barter P.J. Effects of serum amyloid A protein (SAA) on composition, size, and density of high density lipoproteins in subjects with myocardial infarction. J. Lipid. Res. 26 12 (1985) 1389-1398
91. Steinmetz A., Hocke G., Saile R., Puchois P., and Fruchart J.C. Influence of serum amyloid A on cholesterol esterification in human plasma. Biochim. Biophys. Acta 1006 2 (1989) 173-178
92. Xu L., Badolato R., Murphy W.J., Longo D.L., Anver M., Hale S., et al. A novel biologic function of serum amyloid A. Induction of T lymphocyte migration and adhesion. J. Immunol. 155 3 (1995) 1184-1190
93. Kluve-Beckerman B., Manaloor J.J., and Liepnieks J.J. A pulse-chase study tracking the conversion of macrophage-endocytosed serum amyloid A into extracellular amyloid. Arthritis Rheum. 46 7 (2002) 1905-1913
94. van der Hilst J.C., Simon A., and Drenth J.P. Hereditary periodic fever and reactive amyloidosis. Clin. Exp. Med. 5 3 (2005) 87-98
95. Lachmann H.J., Goodman H.J., Gilbertson J.A., Gallimore J.R., Sabin C.A., Gillmore J.D., et al. Natural history and outcome in systemic AA amyloidosis. N. Engl. J. Med. 356 23 (2007) 2361-2371
96. Tanaka F., Migita K., Honda S., Fukuda T., Mine M., Nakamura T., et al. Clinical outcome and survival of secondary (AA) amyloidosis. Clin. Exp. Rheumatol. 21 3 (2003) 343-346
97. Bohle A., Wehrmann M., Eissele R., von Gise H., Mackensen-Haen S., and Muller C. The long-term prognosis of AA and AL renal amyloidosis and the pathogenesis of chronic renal failure in renal amyloidosis. Pathol. Res. Pract. 189 3 (1993) 316-331
98. Uda H., Yokota A., Kobayashi K., Miyake T., Fushimi H., Maeda A., et al. Two distinct clinical courses of renal involvement in rheumatoid patients with AA amyloidosis. J. Rheumatol. 33 8 (2006) 1482-1487
99. Ando Y., Nakamura M., and Araki S. Transthyretin-related familial amyloidotic polyneuropathy. Arch. Neurol. 62 7 (2005) 1057-1062
100. Holmgren G., Costa P.M., Andersson C., Asplund K., Steen L., Beckman L., et al. Geographical distribution of TTR met30 carriers in northern Sweden: discrepancy between carrier frequency and prevalence rate. J. Med. Genet. 31 5 (1994) 351-354
101. Takahashi K., Sakashita N., Ando Y., Suga M., and Ando M. Late onset type I familial amyloidotic polyneuropathy: presentation of three autopsy cases in comparison with 19 autopsy cases of the ordinary type. Pathol. Int. 47 6 (1997) 353-359
102. Sousa M.M., and Saraiva M.J. Neurodegeneration in familial amyloid polyneuropathy: from pathology to molecular signaling. Prog. Neurobiol. 71 5 (2003) 385-400
103. Yazaki M., Tokuda T., Nakamura A., Higashikata T., Koyama J., Higuchi K., et al. Cardiac amyloid in patients with familial amyloid polyneuropathy consists of abundant wild-type transthyretin. Biochem. Biophys. Res. Commun. 274 3 (2000) 702-706
104. Cornwell III G.G., Murdoch W.L., Kyle R.A., Westermark P., and Pitkanen P. Frequency and distribution of senile cardiovascular amyloid. A clinicopathologic correlation. Am. J. Med. 75 4 (1983) 618-623
105. Kyle R.A., Spittell P.C., Gertz M.A., Li C.Y., Edwards W.D., Olson L.J., et al. The premortem recognition of systemic senile amyloidosis with cardiac involvement. Am. J. Med. 101 4 (1996) 395-400
106. Westermark P., Bergstrom J., Solomon A., Murphy C., and Sletten K. Transthyretin-derived senile systemic amyloidosis: clinicopathologic and structural considerations. Amyloid 10 Suppl. 1 (2003) 48-54
107. Ng B., Connors L.H., Davidoff R., Skinner M., and Falk R.H. Senile systemic amyloidosis presenting with heart failure: a comparison with light chain-associated amyloidosis. Arch. Intern. Med. 165 12 (2005) 1425-1429
108. Hagari Y., Mihara M., Konohana I., Ueki H., Yamamoto O., and Koizumi H. Nodular localized cutaneous amyloidosis: further demonstration of monoclonality of infiltrating plasma cells in four additional Japanese patients. Br. J. Dermatol. 138 4 (1998) 652-654
109. Hamidi Asl K., Liepnieks J.J., Nakamura M., and Benson M.D. Organ-specific (localized) synthesis of Ig light chain amyloid. J. Immunol. 162 9 (1999) 5556-5560
110. Setoguchi M., Hoshii Y., Kawano H., and Ishihara T. Analysis of plasma cell clonality in localized AL amyloidosis. Amyloid 7 1 (2000) 41-45
111. Livneh A., Shtrasburg S., Martin B.M., Baniel J., Gal R., and Pras M. Light chain amyloidosis of the urinary bladder. A site restricted deposition of an externally produced immunoglobulin. J. Clin. Pathol. 54 12 (2001) 920-923
112. Tirzaman O., Wahner-Roedler D.L., Malek R.S., Sebo T.J., Li C.Y., and Kyle R.A. Primary localized amyloidosis of the urinary bladder: a case series of 31 patients. Mayo Clin. Proc. 75 12 (2000) 1264-1268
113. Hui A.N., Koss M.N., Hochholzer L., and Wehunt W.D. Amyloidosis presenting in the lower respiratory tract. Clinicopathologic, radiologic, immunohistochemical, and histochemical studies on 48 cases. Arch. Pathol. Lab. Med. 110 3 (1986) 212-218
114. O'Regan A., Fenlon H.M., Beamis Jr. J.F., Steele M.P., Skinner M., and Berk J.L. Tracheobronchial amyloidosis. The Boston University experience from 1984 to 1999. Medicine (Baltimore) 79 2 (2000) 69-79
115. Rubinow A., Celli B.R., Cohen A.S., Rigden B.G., and Brody J.S. Localized amyloidosis of the lower respiratory tract. Am. Rev. Respir. Dis. 118 3 (1978) 603-611
116. Utz J.P., Swensen S.J., and Gertz M.A. Pulmonary amyloidosis. The Mayo Clinic experience from 1980 to 1993. Ann. Intern. Med. 124 4 (1996) 407-413
117. Thompson L.D., Derringer G.A., and Wenig B.M. Amyloidosis of the larynx: a clinicopathologic study of 11 cases. Mod. Pathol. 13 5 (2000) 528-535
118. Huilgol S.C., Ramnarain N., Carrington P., Leigh I.M., and Black M.M. Cytokeratins in primary cutaneous amyloidosis. Australas J. Dermatol. 39 2 (1998) 81-85
119. Brownstein M.H., and Helwig E.B. The cutaneous amyloidoses. I. Localized forms. Arch. Dermatol. 102 1 (1970) 8-19
120. Moon A.O., Calamia K.T., and Walsh J.S. Nodular amyloidosis: review and long-term follow-up of 16 cases. Arch. Dermatol. 139 9 (2003) 1157-1159
121. Woollons A., and Black M.M. Nodular localized primary cutaneous amyloidosis: a long-term follow-up study. Br. J. Dermatol. 145 1 (2001) 105-109
122. Haggqvist B., Naslund J., Sletten K., Westermark G.T., Mucchiano G., Tjernberg L.O., et al. Medin: an integral fragment of aortic smooth muscle cell-produced lactadherin forms the most common human amyloid. Proc. Natl. Acad. Sci. USA 96 15 (1999) 8669-8674
123. Peng S., Westermark G.T., Naslund J., Haggqvist B., Glennert J., and Westermark P. Medin and medin-amyloid in aging inflamed and non-inflamed temporal arteries. J. Pathol. 196 1 (2002) 91-96
124. Peng S., Larsson A., Wassberg E., Gerwins P., Thelin S., Fu X., et al. Role of aggregated medin in the pathogenesis of thoracic aortic aneurysm and dissection. Lab. Invest. 87 12 (2007) 1195-1205
125. Kyle R.A., Gertz M.A., Greipp P.R., Witzig T.E., Lust J.A., Lacy M.Q., et al. A trial of three regimens for primary amyloidosis: colchicine alone, melphalan and prednisone, and melphalan, prednisone, and colchicine. N. Engl. J. Med. 336 17 (1997) 1202-1207
126. 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. 100 3 (1996) 290-298
127. Palladini G., Perfetti V., Obici L., Caccialanza R., Semino A., Adami F., et al. 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 103 8 (2004) 2936-2938
128. Dhodapkar M.V., Hussein M.A., Rasmussen E., Solomon A., Larson R.A., Crowley J.J., et al. 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 104 12 (2004) 3520-3526
129. Palladini G., Perfetti V., Perlini S., Obici L., Lavatelli F., Caccialanza R., et al. The combination of thalidomide and intermediate-dose dexamethasone is an effective but toxic treatment for patients with primary amyloidosis (AL). Blood 105 7 (2005) 2949-2951
130. Dispenzieri A., Lacy M.Q., Rajkumar S.V., Geyer S.M., Witzig T.E., Fonseca R., et al. Poor tolerance to high doses of thalidomide in patients with primary systemic amyloidosis. Amyloid 10 4 (2003) 257-261
131. Sanchorawala V., Wright D.G., Rosenzweig M., Finn K.T., Fennessey S., Zeldis J.B., et al. Lenalidomide and dexamethasone in the treatment of AL amyloidosis: results of a phase 2 trial. Blood 109 2 (2007) 492-496
132. Wechalekar A.D., Goodman H.J., Lachmann H.J., Offer M., Hawkins P.N., and Gillmore J.D. Safety and efficacy of risk-adapted cyclophosphamide, thalidomide, and dexamethasone in systemic AL amyloidosis. Blood 109 2 (2007) 457-464
133. Hussein M.A., Juturi J.V., Rybicki L., Lutton S., Murphy B.R., and Karam M.A. Etanercept therapy in patients with advanced primary amyloidosis. Med. Oncol. 20 3 (2003) 283-290
134. Nakamura T., Higashi S., Tomoda K., Tsukano M., and Baba S. Efficacy of etanercept in patients with AA amyloidosis secondary to rheumatoid arthritis. Clin. Exp. Rheumatol. 25 4 (2007) 518-522
135. Leung N., Dispenzieri A., Fervenza F.C., Lacy M.Q., Villicana R., Cavalcante J.L., et al. Renal response after high-dose melphalan and stem cell transplantation is a favorable marker in patients with primary systemic amyloidosis. Am. J. Kidney Dis. 46 2 (2005) 270-277
136. Jaccard A., Moreau P., Leblond V., Leleu X., Benboubker L., Hermine O., et al. High-dose melphalan versus melphalan plus dexamethasone for AL amyloidosis. N. Engl. J. Med. 357 11 (2007) 1083-1093
137. Gertz M.A., Lacy M.Q., Dispenzieri A., Hayman S.R., and Kumar S. Transplantation for amyloidosis. Curr. Opin. Oncol. 19 2 (2007) 136-141
138. Comenzo R.L., and Gertz M.A. Autologous stem cell transplantation for primary systemic amyloidosis. Blood 99 12 (2002) 4276-4282
139. Sanchorawala V., Wright D.G., Quillen K., Finn K.T., Dember L.M., Berk J.L., et al. Tandem cycles of high-dose melphalan and autologous stem cell transplantation increases the response rate in AL amyloidosis. Bone Marrow Transplant. 40 6 (2007) 607
140. Herlenius G., Wilczek H.E., Larsson M., and Ericzon B.G. Ten years of international experience with Liver Transplantation for familial amyloidotic polyneuropathy: results from the Familial Amyloidotic Polyneuropathy World Transplant Registry. Transplantation 77 1 (2004) 64-71
141. Takei Y., Gono T., Yazaki M., Ikeda S., Ikegami T., Hashikura Y., et al. Transthyretin-derived amyloid deposition on the gastric mucosa in domino recipients of familial amyloid polyneuropathy liver. Liver Transpl. 13 2 (2007) 215-218
142. Gillmore J.D., Lovat L.B., Persey M.R., Pepys M.B., and Hawkins P.N. Amyloid load and clinical outcome in AA amyloidosis in relation to circulating concentration of serum amyloid A protein. Lancet 358 9275 (2001) 24-29
143. Livneh A., Zemer D., Langevitz P., Shemer J., Sohar E., and Pras M. Colchicine in the treatment of AA and AL amyloidosis. Semin. Arthritis Rheum. 23 3 (1993) 206-214
144. Tojo K., Sekijima Y., Kelly J.W., and Ikeda S. Diflunisal stabilizes familial amyloid polyneuropathy-associated transthyretin variant tetramers in serum against dissociation required for amyloidogenesis. Neurosci. Res. 56 4 (2006) 441-449
145. Colon W., and Kelly J.W. Partial denaturation of transthyretin is sufficient for amyloid fibril formation in vitro. Biochemistry 31 36 (1992) 8654-8660
146. Hammarstrom P., Jiang X., Hurshman A.R., Powers E.T., and Kelly J.W. Sequence-dependent denaturation energetics: a major determinant in amyloid disease diversity. Proc. Natl. Acad. Sci. USA 99 Suppl. 4 (2002) 16427-16432
147. Pepys M.B., Herbert J., Hutchinson W.L., Tennent G.A., Lachmann H.J., Gallimore J.R., et al. Targeted pharmacological depletion of serum amyloid P component for treatment of human amyloidosis. Nature 417 6886 (2002) 254-259
148. Gervais F., Chalifour R., Garceau D., Kong X., Laurin J., McLaughlin R., et al. Glycosaminoglycan mimetics: a therapeutic approach to cerebral amyloid angiopathy. Amyloid 8 Suppl. 1 (2001) 28-35
149. Dember L.M., Hawkins P.N., Hazenberg B.P., Gorevic P.D., Merlini G., Butrimiene I., et al. Eprodisate for the treatment of renal disease in AA amyloidosis. N. Engl. J. Med. 356 23 (2007) 2349-2360
150. Hrncic R., Wall J., Wolfenbarger D.A., Murphy C.L., Schell M., Weiss D.T., et al. Antibody-mediated resolution of light chain-associated amyloid deposits. Am. J. Pathol. 157 4 (2000) 1239-1246
151. Nikolic W.V., Bai Y., Obregon D., Hou H., Mori T., Zeng J., et al. Transcutaneous beta-amyloid immunization reduces cerebral beta-amyloid deposits without T cell infiltration and microhemorrhage. Proc. Natl. Acad. Sci. USA 104 7 (2007) 2507-2512
152. Mayr M., and Dickenmann M.J. [Further evidence for the renoprotective effect of ACE inhibitors: ramipril protects against the progression of chronic renal insufficiency in non-diabetic nephropathy with nephrotic proteinuria]. Schweiz Med. Wochenschr. 130 13 (2000) 491
153. Mathew V., Chaliki H., and Nishimura R.A. Atrioventricular sequential pacing in cardiac amyloidosis: an acute Doppler echocardiographic and catheterization hemodynamic study. Clin. Cardiol. 20 8 (1997) 723-725
154. Caputa C.B., and Salama A.I. The Amyloid Proteins of Alzheimer's Disease as Potential Targets for Drug Therapy (Part 1). Microbiology of Aging (1989), Pergamon Press 451-461