22. Primary Systemic Amyloidosis (AL)

  1. Hussain I. Saba MD, PHD2 and
  2. Ghulam J. Mufti MB, DM, FRCP, FRCPATH3
  1. Efstathios Kastritis and
  2. Meletios Athanasios Dimopoulos

Published Online: 24 MAR 2011

DOI: 10.1002/9781444394016.ch22

Advances in Malignant Hematology

Advances in Malignant Hematology

How to Cite

Kastritis, E. and Athanasios Dimopoulos, M. (2011) Primary Systemic Amyloidosis (AL), in Advances in Malignant Hematology (eds H. I. Saba and G. J. Mufti), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781444394016.ch22

Editor Information

  1. 2

    James A. Haley Veterans' Hospital, H. Lee Moffitt Cancer Center and Research Institute, University of South Florida College of Medicine, Tampa, FL, USA

  2. 3

    Department of Haematological Medicine, Guy's and St Thomas' School of Medicine, King's College Hospital, London, UK

Author Information

  1. Department of Clinical Therapeutics, University of Athens School of Medicine, Athens, Greece

Publication History

  1. Published Online: 24 MAR 2011
  2. Published Print: 16 APR 2011

ISBN Information

Print ISBN: 9781405196260

Online ISBN: 9781444394016

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Keywords:

  • immunoglobulins;
  • bortezomib;
  • lenalidomide;
  • thalidomide;
  • high-dose melphalan;
  • transplantation;
  • multiple myeloma;
  • congestive heart failure;
  • nephrotic syndrome;
  • peripheral neuropathy

Summary

Primary systemic immunoglobulin light chain amyloidosis (AL) results from the accumulation of amyloid fibrils that are composed of a monoclonal immunoglobulin light chain that is produced by a plasma cell clone. Almost every organ can be involved, resulting in multisystemic symptoms and signs. Diagnosis may be difficult and requires a certain degree of suspicion. Treatment is challenging and most patients may be quite frail. Careful assessment of the organ involvement and prognosis is needed before planning the treatment strategy. Supportive care is critical for AL patients. The goal of treatment is the reduction of light chain production. Conventional chemotherapy may be effective and recently introduced novel agents expand treatment options in patients who relapse or are not candidates for high-dose melphalan with autologous stem cell transplant (HDM-ASCT). In experienced centers, HDM-ASCT in patients deemed capable of tolerating the procedure is associated with high response rates with modest treatment-related mortality.