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Assessing Response and Prognosis in AL Amyloidosis

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Amyloidosis

Part of the book series: Contemporary Hematology ((CH))

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Abstract

Advances in assessing response and assigning risk to patients with AL amyloidosis have been made in the past several decades. Historically the most important predictor of overall survival has been the presence and extent of cardiac involvement. Most recently there have been advances for better stratifying cardiac risk; other risk factors have also been identified. Methods for assessing clinical response criteria have also improved with readily available means to measure changes of the serum immunoglobulin free light chain. These developments have been integral for improving outcomes for these patients.

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References

  1. Kyle RA, Greipp PR. Primary systemic amyloidosis: comparison of melphalan and prednisone versus placebo. Blood 1978;52:818–27.

    PubMed  CAS  Google Scholar 

  2. Gertz MA, Lacy MQ, Dispenzieri A, Hayman SR. Amyloidosis. Best Pract Res Clin Haematol. 2005;18:709–27.

    Article  PubMed  CAS  Google Scholar 

  3. Benson MD. Treatment of AL amyloidosis with melphalan, prednisone, and colchicine. Arthritis rheum. 1986;29:683–7.

    Article  PubMed  CAS  Google Scholar 

  4. Gertz MA, Kyle RA, Greipp PR. Response rates and survival in primary systemic amyloidosis. Blood 1991;77:257–62.

    PubMed  CAS  Google Scholar 

  5. Kyle RA, Gertz MA, Greipp PR, et al. Long-term survival (10 years or more) in 30 patients with primary amyloidosis. Blood 1999;93:1062–6.

    PubMed  CAS  Google Scholar 

  6. Kyle RA, Gertz MA, Greipp PR, et al. A trial of three regimens for primary amyloidosis: colchicine alone, melphalan and prednisone, and melphalan, prednisone, and colchicine. N Engl J Med. 1997;336:1202–7.

    Article  PubMed  CAS  Google Scholar 

  7. Comenzo RL, Vosburgh E, Falk RH, et al. Dose-intensive melphalan with blood stem-cell support for the treatment of AL (amyloid light-chain) amyloidosis: survival and responses in 25 patients. Blood 1998;91:3662–70.

    PubMed  CAS  Google Scholar 

  8. Moreau P, Leblond V, Bourquelot P, et al. Prognostic factors for survival and response after high-dose therapy and autologous stem cell transplantation in systemic AL amyloidosis: a report on 21 patients. Br J Haematol. 1998;101:766–9.

    Article  PubMed  CAS  Google Scholar 

  9. Gertz MA, Comenzo R, Falk RH, et al. Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL): a consensus opinion from the 10th International Symposium on Amyloid and Amyloidosis, Tours, France, 18–22 April 2004. Am J Hematol. 2005;79:319–28.

    Article  PubMed  Google Scholar 

  10. Gertz MA, Kyle RA. Primary systemic amyloidosis – a diagnostic primer. Mayo Clin Proc. 1989;64:1505–19.

    Article  PubMed  CAS  Google Scholar 

  11. Lachmann HJ, Gallimore R, Gillmore JD, et al. Outcome in systemic AL amyloidosis in relation to changes in concentration of circulating free immunoglobulin light chains following chemotherapy. Br J Haematol. 2003;122:78–84.

    Article  PubMed  CAS  Google Scholar 

  12. Dispenzieri A, Lacy MQ, Katzmann JA, et al. Absolute values of immunoglobulin free light chains are prognostic in patients with primary systemic amyloidosis undergoing peripheral blood stem cell transplantation. Blood 2006;107:3378–83.

    Article  PubMed  CAS  Google Scholar 

  13. Sanchorawala V, Seldin DC, Magnani B, Skinner M, Wright DG. Serum free light-chain responses after high-dose intravenous melphalan and autologous stem cell transplantation for AL (primary) amyloidosis. Bone Marrow Transplant. 2005;36:597–600.

    Article  PubMed  CAS  Google Scholar 

  14. Palladini G, Lavatelli F, Russo P, et al. Circulating amyloidogenic free light chains and serum N-terminal natriuretic peptide type B decrease simultaneously in association with improvement of survival in AL. Blood 2006;107:3854–8.

    Article  PubMed  CAS  Google Scholar 

  15. Palladini G, Perfetti V, Obici L, 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 2004;103:2936–8.

    Article  PubMed  CAS  Google Scholar 

  16. Skinner M, Sanchorawala V, Seldin DC, et al. High-dose melphalan and autologous stem-cell transplantation in patients with AL amyloidosis: an 8-year study. Ann Intern Med. 2004;140:85–93.

    PubMed  CAS  Google Scholar 

  17. Cohen AD, Zhou P, Chou J, et al. Risk-adapted autologous stem cell transplantation with adjuvant dexamethasone +/– thalidomide for systemic light-chain amyloidosis: results of a phase II trial. Br J Haematol. 2007;139:224–33.

    Article  PubMed  CAS  Google Scholar 

  18. Palladini G, Campana C, Klersy C, et al. Serum N-terminal pro-brain natriuretic peptide is a sensitive marker of myocardial dysfunction in AL amyloidosis. Circulation 2003;107:2440–5.

    Article  PubMed  CAS  Google Scholar 

  19. Dispenzieri A, Gertz MA, Kyle RA, et al. Serum cardiac troponins and N-terminal pro-brain natriuretic peptide: a staging system for primary systemic amyloidosis. J Clin Oncol. 2004;22:3751–7.

    Article  PubMed  CAS  Google Scholar 

  20. Dispenzieri A, Gertz MA, Kyle RA, 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;104:1881–7.

    Article  PubMed  CAS  Google Scholar 

  21. Jaccard A, Moreau P, Leblond V, et al. High-dose melphalan versus melphalan plus dexamethasone for AL amyloidosis. N Engl J Med. 2007;357:1083–93.

    Article  PubMed  CAS  Google Scholar 

  22. Gertz MA, Lacy MQ, Dispenzieri A, et al. Effect of hematologic response on outcome of patients undergoing transplantation for primary amyloidosis: importance of achieving a complete response. Haematologica 2007;92:1415–8.

    Article  PubMed  Google Scholar 

  23. Palladini G, Russo P, Nuvolone M, et al. Treatment with oral melphalan plus dexamethasone produces long-term remissions in AL amyloidosis. Blood 2007;110:787–8.

    Article  PubMed  CAS  Google Scholar 

  24. Sanchorawala V, Skinner M, Quillen K, Finn KT, Doros G, Seldin DC. Long-term outcome of patients with AL amyloidosis treated with high-dose melphalan and stem-cell transplantation. Blood 2007;110:3561–3.

    Article  PubMed  CAS  Google Scholar 

  25. Kyle RA, Greipp PR, O’Fallon WM. Primary systemic amyloidosis: multivariate analysis for prognostic factors in 168 cases. Blood 1986;68:220–4.

    PubMed  CAS  Google Scholar 

  26. Kyle RA, Gertz MA. Primary systemic amyloidosis: clinical and laboratory features in 474 cases. Semin Hematol. 1995;32:45–59.

    PubMed  CAS  Google Scholar 

  27. Kyle RA, Bayrd ED. Amyloidosis: review of 236 cases. Medicine 1975;54:271–99.

    Article  PubMed  CAS  Google Scholar 

  28. Kyle RA, Greipp PR, Garton JP, Gertz MA. Primary systemic amyloidosis. Comparison of melphalan/prednisone versus colchicine. Am J Med. 1985;79:708–16.

    Article  PubMed  CAS  Google Scholar 

  29. Cohen AS, Rubinow A, Anderson JJ, Skinner M, Mason JH, Libbey C, Kayne H. Survival of patients with primary (AL) amyloidosis. Colchicine-treated cases from 1976 to 1983 compared with cases seen in previous years (1961 to 1973). Am J Med. 1987;82:1182–90.

    Article  PubMed  CAS  Google Scholar 

  30. Gertz MA, Kyle RA. Amyloidosis: prognosis and treatment. Semin Arthritis Rheum. 1994;24:124–38.

    Article  PubMed  CAS  Google Scholar 

  31. Gertz MA, Lacy MQ, Dispenzieri A. Amyloidosis: recognition, confirmation, prognosis, and therapy. Mayo Clin Proc. 1999;74:490–4.

    PubMed  CAS  Google Scholar 

  32. Cueto-Garcia L, Reeder GS, Kyle RA, et al. Echocardiographic findings in systemic amyloidosis: spectrum of cardiac involvement and relation to survival. J Am Coll Cardiol. 1985;6:737–43.

    Article  PubMed  CAS  Google Scholar 

  33. Klein AL, Hatle LK, Taliercio CP, et al. Prognostic significance of Doppler measures of diastolic function in cardiac amyloidosis. A Doppler echocardiography study. Circulation 1991;83:808–16.

    Article  PubMed  CAS  Google Scholar 

  34. Oh JK, Tajik AJ, Edwards WD, Bresnahan JF, Kyle RA. Dynamic left ventricular outflow tract obstruction in cardiac amyloidosis detected by continuous-wave Doppler echocardiography. Am J Cardiol. 1987;59:1008–10.

    Article  PubMed  CAS  Google Scholar 

  35. Klein AL, Hatle LK, Burstow DJ, et al. Comprehensive Doppler assessment of right ventricular diastolic function in cardiac amyloidosis. J Am Coll Cardiol. 1990;15:99–108.

    Article  PubMed  CAS  Google Scholar 

  36. Ghio S, Perlini S, Palladini G, et al. Importance of the echocardiographic evaluation of right ventricular function in patients with AL amyloidosis. Eur J Heart Fail. 2007;9: 808–13.

    Article  PubMed  Google Scholar 

  37. Patel AR, Dubrey SW, Mendes LA, et al. Right ventricular dilation in primary amyloidosis: an independent predictor of survival. Am J Cardiol. 1997;80:486–92.

    Article  PubMed  CAS  Google Scholar 

  38. Modesto KM, Dispenzieri A, Cauduro SA, et al. Left atrial myopathy in cardiac amyloidosis: implications of novel echocardiographic techniques. Eur Heart J. 2005;26:173–9.

    Article  PubMed  Google Scholar 

  39. Koyama J, Ray-Sequin PA, Falk RH. Longitudinal myocardial function assessed by tissue velocity, strain, and strain rate tissue Doppler echocardiography in patients with AL (primary) cardiac amyloidosis. Circulation 2003;107:2446–52.

    Article  PubMed  Google Scholar 

  40. Bellavia D, Abraham TP, Pellikka PA, et al. Detection of left ventricular systolic dysfunction in cardiac amyloidosis with strain rate echocardiography. J Am Soc Echocardiogr. 2007;20:1194–202.

    Article  PubMed  Google Scholar 

  41. Bellavia D, Pellikka PA, Abraham TP, et al. Evidence of impaired left ventricular systolic function by Doppler myocardial imaging in patients with systemic amyloidosis and no evidence of cardiac involvement by standard two-dimensional and Doppler echocardiography. Am J Cardiol. 2008;101:1039–45.

    Article  PubMed  Google Scholar 

  42. Bellavia D, Pellikka PA, Abraham TP, et al. ‘Hypersynchronisation’ by tissue velocity imaging in patients with cardiac amyloidosis. Heart 2009;95:234–40.

    Article  PubMed  CAS  Google Scholar 

  43. Maceira AM, Prasad SK, Hawkins PN, Roughton M, Pennell DJ. Cardiovascular magnetic resonance and prognosis in cardiac amyloidosis. J Cardiovasc Magn Reson. 2008;10:54.

    Article  PubMed  Google Scholar 

  44. Ruberg FL, Appelbaum E, Davidoff R, et al. Diagnostic and prognostic utility of cardiovascular magnetic resonance imaging in light-chain cardiac amyloidosis. Am J Cardiol. 2009;103:544–9.

    Article  PubMed  Google Scholar 

  45. Palladini G, Malamani G, Co F, et al. Holter monitoring in AL amyloidosis: prognostic implications. Pacing Clin Electrophysiol. 2001;24:1228–33.

    Article  PubMed  CAS  Google Scholar 

  46. Dispenzieri A, Kyle RA, Gertz MA, et al. Survival in patients with primary systemic amyloidosis and raised serum cardiac troponins. Lancet 2003;361:1787–9.

    Article  PubMed  CAS  Google Scholar 

  47. Gertz MA, Kyle RA, Greipp PR. The plasma cell labeling index: a valuable tool in primary systemic amyloidosis. Blood 1989;74:1108–11.

    PubMed  CAS  Google Scholar 

  48. Pardanani A, Witzig TE, Schroeder G, et al. Circulating peripheral blood plasma cells as a prognostic indicator in patients with primary systemic amyloidosis. Blood 2003;101: 827–30.

    Article  PubMed  CAS  Google Scholar 

  49. Kyle RA, Greipp PR. Amyloidosis (AL). Clinical and laboratory features in 229 cases. Mayo Clin Proc. 1983;58:665–83.

    PubMed  CAS  Google Scholar 

  50. Gertz MA, Kyle RA. Prognostic value of urinary protein in primary systemic amyloidosis (AL). Am J Clin Pathol. 1990;94:313–7.

    PubMed  CAS  Google Scholar 

  51. Bryce AH, Ketterling R, Gertz MA, et al. Association of translocation t(11;14) with survival in patients with light chain (AL) amyloidosis [Abstract 8549]. J Clin Oncol. 2008;26.

    Google Scholar 

  52. Bochtler T, Hegenbart U, Cremer FW, 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;111:4700–5.

    Article  PubMed  CAS  Google Scholar 

  53. Gertz MA, Lacy MQ, Dispenzieri A, Hayman SR, Kumar S. Transplantation for amyloidosis. Curr Opin Oncol. 2007;19:136–41.

    Article  PubMed  CAS  Google Scholar 

  54. Gertz MA, Kyle RA, Greipp PR, Katzmann JA, O’Fallon WM. Beta 2-microglobulin predicts survival in primary systemic amyloidosis. Am J Med. 1990;89:609–14.

    Article  PubMed  CAS  Google Scholar 

  55. Hazenberg BP, van Rijswijk MH, Lub-de Hooge MN, et al. Diagnostic performance and prognostic value of extravascular retention of 123I-labeled serum amyloid P component in systemic amyloidosis. J Nucl Med. 2007;48:865–72.

    Article  PubMed  CAS  Google Scholar 

  56. Hachulla E, Maulin L, Deveaux M, et al. Prospective and serial study of primary amyloidosis with serum amyloid P component scintigraphy: from diagnosis to prognosis. Am J Med. 1996;101:77–87.

    Article  PubMed  CAS  Google Scholar 

  57. Chamarthi B, Dubrey SW, Cha K, Skinner M, Falk RH. Features and prognosis of exertional syncope in light-chain associated AL cardiac amyloidosis. Am J Cardiol. 1997;80:1242–5.

    Article  PubMed  CAS  Google Scholar 

  58. Dubrey SW, Cha K, Anderson J, et al. The clinical features of immunoglobulin light-chain (AL) amyloidosis with heart involvement. QJM. 1998;91:141–57.

    Article  PubMed  CAS  Google Scholar 

  59. Lebovic D, Hoffman J, Levine BM, et al. 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;143:369–73.

    Article  PubMed  CAS  Google Scholar 

  60. Gertz MA, Kyle RA, Greipp PR. Hyposplenism in primary systemic amyloidosis. Ann Intern Med. 1983;98:475–7.

    PubMed  CAS  Google Scholar 

  61. Kumar S, Dispenzieri A, Lacy MQ, et al. Serum uric acid: novel prognostic factor in primary systemic amyloidosis. Mayo Clin Proc. 2008;83:297–303.

    Article  PubMed  CAS  Google Scholar 

  62. Kumar S, Dispenzieri A, Lacy MQ, et al. Improved survival in light chain amyloidosis. Amyloid 2010;17(Suppl 1):89.

    Google Scholar 

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Acknowledgments

This work was supported in part by the Hematologic Malignancies Fund.

Financial support: AD is supported in part by grants CA125614, CA062242, CA111345, and CA107476 from the National Cancer Institute.

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Authors and Affiliations

  1. Department of Medicine and Laboratory Medicine and Pathology, Mayo Clinic College of Medicine, Rochester, MN, USA

    Angela Dispenzieri MD

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  1. Angela Dispenzieri MD
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Editors and Affiliations

  1. Mayo Clinic, First Street SW. 200, Rochester, 55905, Minnesota, USA

    Morie A. Gertz

  2. Mayo Clinic, First Street SW. 200, Rochester, 55905, Minnesota, USA

    S. Vincent Rajkumar

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Dispenzieri, A. (2010). Assessing Response and Prognosis in AL Amyloidosis. In: Gertz, M., Rajkumar, S. (eds) Amyloidosis. Contemporary Hematology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-60761-631-3_6

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  • DOI: https://doi.org/10.1007/978-1-60761-631-3_6

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