Co-deposition of amyloidogenic immunoglobulin light and heavy chains in localized pulmonary amyloidosis.
Localized pulmonary amyloidosis is a rare condition whose pathogenesis is insufficiently understood. In the present study, we report a case of localized pulmonary amyloidosis associated with lung-restricted lymphoplasmacytoid lymphoma, monoclonal for immunoglobulin (Ig) G lambda (lambda). Biochemical microtechniques have been applied for extraction, purification, and characterization of amyloid proteins. Surprisingly, chemical analysis of these proteins revealed a not-previously-described case of combined deposits containing Ig fragments of gamma heavy chain (variable domain) and lambda light chain (constant domain). In view of the absence of circulating monoclonal Ig, this case supports the hypothesis that localized amyloid is formed by local plasmacytoid cells.
Kaplan
Batia
B
Heller Institute of Medical Research, Sheba Medical Center, Tel Hashomer, 52621, Israel. kaplanb@sheba.health.gov.il
Martin
Brian M
BM
Boykov
Olga
O
Gal
Rivka
R
Pras
Mordechai
M
Shechtman
Itzhak
I
Saute
Milton
M
Kramer
Mordechai R
MR
D000368:Aged; D000682:Amyloid / Q000737:chemistry*; D000686:Amyloidosis / Q000150:complications / Q000473:pathology*; D015153:Blotting, Western; D004591:Electrophoresis, Polyacrylamide Gel; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000378:metabolism*; D007146:Immunoglobulin lambda-Chains / Q000378:metabolism*; D007150:Immunohistochemistry; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000150:complications / Q000473:pathology*; D008171:Lung Diseases / Q000150:complications / Q000473:pathology*; D008297:Male; D011014:Pneumonia / Q000473:pathology
D000682:Amyloid; D007143:Immunoglobulin Heavy Chains; D007146:Immunoglobulin lambda-Chains