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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, Author, and Disclosure 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
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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.





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