0

The full content of Annals is available to subscribers

Subscribe/Learn More  >
Reviews |

Monoclonal Immunoglobulin Deposition Disease: Light Chain and Light and Heavy Chain Deposition Diseases and Their Relation to Light Chain Amyloidosis: Clinical Features, Immunopathology, and Molecular Analysis

Joel N. Buxbaum, MD; Joseph V. Chuba, PhD; Gerard C. Hellman, MD; Alan Solomon, MD; and Gloria R. Gallo, MD
[+] Article and Author Information

Grant Support: By Merit Review research funds from the Veteran's Administration, a grant-in-aid from the New York/New Jersey chapter of the National Kidney Foundation, NIH grant CA 10056, an Ina M. Barger Memorial Grant for Cancer Research from the American Cancer Society, IM-430.

Requests for Reprints: J. Buxbaum, MD, Research Service, NYVA Medical Center, 408 First Avenue, New York, NY 10010.

Current Author Addresses: Dr. Buxbaum: Research Service, NYVA Medical Center, 408 First Avenue, New York, NY 10010.

Drs. Gallo and Chuba: Department of Pathology, New York University Medical Center, 550 First Avenue, New York, NY 10016.

Dr. Solomon: Department of Medicine, University of Tennessee Medical Center at Knoxville, Knoxville, TN 37920.

Dr. Hellman: Department of Medicine, Lenox Hill Hospital, New York, NY 10021.


Ann Intern Med. 1990;112(6):455-464. doi:10.7326/0003-4819-76-3-112-6-455
Text Size: A A A

Monoclonal immunoglobulin deposition occurs in tissues as Congo Red binding fibrils in light chain amyloidosis, as less structured deposits in light chain deposition disease, and as similar but distinct deposits in light and heavy chain deposition disease. The nonamyloid forms were found in 13 patients who had evidence of plasmacytic dyscrasia by the immunohistochemical detection of immunoglobulin light chains of kappa or lambda class (with or without staining for a single heavy chain isotype) and by the absence of amyloid P component in tissue sections that did not show the birefringence characteristic of amyloid after Congo Red staining. All but two of the patients presented with proteinuria with or without azotemia. Clinical syndromes involving other organ systems were less common but occasionally severe. Four patients had overt multiple myeloma. Three others had hypercalcemia and mild bone marrow plasmacytosis but no lytic lesions. Analyses of immunoglobulin synthesis in bone marrow cells from seven patients showed excess light chains in all and incomplete light chains or heavy chain fragments in six, regardless of whether an intact monoclonal protein or related subunit was in the serum or urine. The fibrillar (amyloidotic) and nonfibrillar forms of monoclonal immunoglobulin deposition occur either in overt multiple myeloma or in the course of less neoplastically aggressive plasmacytic dyscrasias. Bone marrow cells from patients with either type produce immunoglobulin fragments that are related to those deposited in the affected tissues.

Figures

Tables

References

Letters

NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Comments

Submit a Comment
Submit a Comment

Summary for Patients

Clinical Slide Sets

Terms of Use

The In the Clinic® slide sets are owned and copyrighted by the American College of Physicians (ACP). All text, graphics, trademarks, and other intellectual property incorporated into the slide sets remain the sole and exclusive property of the ACP. The slide sets may be used only by the person who downloads or purchases them and only for the purpose of presenting them during not-for-profit educational activities. Users may incorporate the entire slide set or selected individual slides into their own teaching presentations but may not alter the content of the slides in any way or remove the ACP copyright notice. Users may make print copies for use as hand-outs for the audience the user is personally addressing but may not otherwise reproduce or distribute the slides by any means or media, including but not limited to sending them as e-mail attachments, posting them on Internet or Intranet sites, publishing them in meeting proceedings, or making them available for sale or distribution in any unauthorized form, without the express written permission of the ACP. Unauthorized use of the In the Clinic slide sets will constitute copyright infringement.

Toolkit

Buy Now

to gain full access to the content and tools.

Want to Subscribe?

Learn more about subscription options

Advertisement
Related Articles
Related Point of Care
Topic Collections

Buy Now

to gain full access to the content and tools.

Want to Subscribe?

Learn more about subscription options

Forgot your password?
Enter your username and email address. We'll send you a reminder to the email address on record.
(Required)
(Required)