Immunoglobulin-Related Amyloidosis Follow-up

  • Author: Slavomir Urbancek, MD, PhD; Chief Editor: Emmanuel C Besa, MD  more...
Updated: Jan 29, 2015

Further Outpatient Care

A hematologist with experience in administering chemotherapy should care for patients with L chain–type amyloidosis on an ongoing basis.



Complications of L chain–type amyloidosis reflect the organ systems involved. The most severe complication of systemic L chain–type amyloidosis is extensive cardiac deposition, with consequent congestive heart failure, arrhythmias, or both. Cardiac involvement eventually occurs in most patients and appears to be the cause of death in more than 50% of patients with L chain–type amyloidosis.



The prognosis for patients with L chain–type amyloidosis depends largely on the specificity of the tissue deposition. Any organ can be involved, with symptoms and physical findings reflecting the pattern of anatomic compromise.

Patients with clinical cardiac involvement have the worst prognosis, with a median survival rate of 6 months. In these patients, increased troponin is associated with worse left ventricular and left atrial functions. Increased troponin at baseline strongly predicts all-cause mortality.[25]

Median overall survival was 29 months in a retrospective study of 63 patients with L chain–type amyloidosis, of which 32 (51%) presented with cardiac amyloid involvement. Overall survival did not differ between patients with or without cardiac involvement. During a median follow-up of 12.7 months, 38 (60%) patients died.[26]

Patients with involvement limited to the peripheral nerves have the longest survival. Other favorable prognostic features include a small number of clonal plasma cells in the bone marrow and normal renal function.

In the absence of chemotherapy, systemic L chain–type amyloidosis is always progressive. A subgroup of cases respond to chemotherapy with temporary resorption of amyloid fibrils and improvement of end-organ function.

In a retrospective study of 146 patients with L chain–type amyloidosis who relapsed after treatment wih chemotherapy and autologous stem cell transplant, median overall survival and 5-year overall survival from the time of post-transplant relapse were 51.7 months and 39%, respectively.[27]

Contributor Information and Disclosures

Slavomir Urbancek, MD, PhD Head, Department of Dermatology, FD Roosevelt Hospital, Slovakia; Member of executive commitee - Slovak Dermatovenereological Society

Slavomir Urbancek, MD, PhD is a member of the following medical societies: American Academy of Dermatology, European Academy of Dermatology and Venereology, Slovakian Dermatovenerological Society, Slovak Society of Allergology and Clinical Immunology

Disclosure: Received honoraria from Abbott for speaking and teaching; Received honoraria from Pfizer for none; Received honoraria from Janssen for speaking and teaching; Received honoraria from MSD for speaking and teaching; Received consulting fee from Roche for speaking and teaching.


Daniel R Jacobson, MD Professor of Medicine, Boston University School of Medicine; Chief of Oncology, Veterans Affairs Boston Healthcare System

Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Carol A Bogdan, MD Consultant in Hematology-Oncology, Myrtle Beach, SC

Disclosure: Nothing to disclose.

Joel N Buxbaum, MD Professor, Department of Molecular and Experimental Medicine, The Scripps Research Institute

Joel N Buxbaum, MD is a member of the following medical societies: American Association for the Advancement of Science, American Society for Clinical Investigation, American Society of Human Genetics, Association of American Physicians, Association of Black Cardiologists, Henry Kunkel Society

Disclosure: Received consulting fee from Foldrx pharmaceuticals for consulting; Received consulting fee from Isis pharmaceuticals for consulting; Received consulting fee from alnylam pharmaceutical for consulting; Received consulting fee from Pfizer Pharmaceuticals for consulting.

Pere Gascon, MD, PhD Professor and Director, Division of Medical Oncology, Institute of Hematology and Medical Oncology, IDIBAPS, University of Barcelona Faculty of Medicine, Spain

Pere Gascon, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, New York Academy of Medicine, New York Academy of Sciences, Sigma Xi

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Lawrence H Brent, MD Associate Professor of Medicine, Jefferson Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, American College of Rheumatology

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Janssen<br/>Serve(d) as a speaker or a member of a speakers bureau for: Genentech; Pfizer; Mallinckrodt.

Chief Editor

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Robert E Wolf, MD, PhD Professor Emeritus, Department of Medicine, Louisiana State University School of Medicine in Shreveport; Chief, Rheumatology Section, Medical Service, Overton Brooks Veterans Affairs Medical Center

Robert E Wolf, MD, PhD is a member of the following medical societies: American College of Rheumatology, Arthritis Foundation, Society for Leukocyte Biology

Disclosure: Nothing to disclose.

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The relationship among light chain–type amyloidosis (AL), the other monoclonal plasma cell disorders, and the other amyloidoses. Ig = immunoglobulin; MGUS = monoclonal gammopathy of undetermined significance.
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