A case of gamma 3 heavy chain disease with vacuolated plasma cells: a clinical, immunological, and ultrastructural study.
A patient with lambda Bence-Jones proteinuria, Waldenström's macroglobulinaemia, and Franklin's disease (gamma HCD), but without clinical evidence of a lymphoproliferative disorder, is presented. The serum contained two distinct immunoglobulin abnormalities: a monoclonal immunoglobulin M (IgM) of lambda type, and a protein fragment which was immunologically related to immunoglobulin G (IgG) and devoid of light chain activity. This gamma HCD protein belongs to the gamma 3 subclass with a molecular weight of approximately 60,000 daltons. The urine contained a Bence-Jones lambda protein as well as the gamma HCD fragment. The two paraproteins were probably secreted by two different malignant clones. Ultrastructural study revealed pathological vacuolated plasma cells of a sort that has hitherto been principally described in association with micron HCD. The mechanism of the intracellular storage of pathological immunoglobulins is discussed in the light of the ultrastructural study.
D000368:Aged; D001536:Bence Jones Protein / Q000652:urine; D005260:Female; D006362:Heavy Chain Disease / Q000150:complications* / Q000276:immunology / Q000473:pathology; D006801:Humans; D007143:Immunoglobulin Heavy Chains; D007075:Immunoglobulin M / Q000032:analysis; D007142:Immunoglobulin gamma-Chains; D009940:Organoids / Q000648:ultrastructure*; D010950:Plasma Cells / Q000648:ultrastructure*; D011507:Proteinuria / Q000150:complications; D014617:Vacuoles / Q000648:ultrastructure*; D008258:Waldenstrom Macroglobulinemia / Q000150:complications
D007143:Immunoglobulin Heavy Chains; D007075:Immunoglobulin M; D007142:Immunoglobulin gamma-Chains; D001536:Bence Jones Protein