Celluar immunoglobulins in human gamma- and alpha-heavy chain diseases.
Proliferating cells from twenty-four patients with alpha- or gamma-heavy chain disease (HCD) were studied by direct immunofluorescence and in several cases by biosynthesis experiments with 14C-amino acid incorporation. In twenty-two patients, the cells contained the HCD proteins only and no light chain synthesis could be detected. Conversely, apparently non-secreted monotypic light chains were found in one case of gamma-HCD and one case of alpha-HCD. The proportion of proliferating cells containing cytoplasmic heavy chains, their appearance and the presence or not of surface heavy chains showed great variation from patient to patient. In some cases, the proliferation predominantly affected either plasma cells or lymphocytes whereas in others the disease seemed to correspond to a proliferation of HCD protein-bearing lymphocytes with persistent maturation into plasma cells. Large cell lymphomas supervening on alpha-HCD belonged to the same proliferating clone as the clone secreting the HCD protein, as shown by surface markers and biosynthesis experiments which demonstrated synthesis but no secretion of HCD proteins. In one patient with gamma-HCD, the cells carried surface gamma and delta chains.
D000328:Adult; D000368:Aged; D002648:Child; D006362:Heavy Chain Disease / Q000276:immunology*; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000032:analysis*; D007147:Immunoglobulin Light Chains / Q000032:analysis* / Q000096:biosynthesis; D007137:Immunoglobulin alpha-Chains / Q000032:analysis* / Q000096:biosynthesis; D007142:Immunoglobulin gamma-Chains / Q000032:analysis* / Q000096:biosynthesis; D008214:Lymphocytes / Q000276:immunology; D016403:Lymphoma, Large B-Cell, Diffuse / Q000276:immunology; D008875:Middle Aged; D010950:Plasma Cells / Q000276:immunology; D011947:Receptors, Antigen, B-Cell / Q000032:analysis
D007143:Immunoglobulin Heavy Chains; D007147:Immunoglobulin Light Chains; D007137:Immunoglobulin alpha-Chains; D007142:Immunoglobulin gamma-Chains; D011947:Receptors, Antigen, B-Cell