Evidence for immune complexes involving anti-lymphocyte antibodies associated with hypocomplementaemia in chronic lymphocytic leukaemia (CLL).
Unmeasurable total haemolytic complement (C) was observed in serum of a patient with untreated chronic lymphocytic leukaemia and recurrent non-hereditary angioedema. Analysis of C components immunochemically demonstrated a marked reduction of C1q and C1s inhibitor, undetectable C1r, C1s and an elevated B. Haemolytic C1, C4 and C2 were less than 5 percent of normal, functional C1s inhibitor was absent. Cryoglobulin and C1q precipitins were present in the serum. Of special interest was the presence of high levels of cold-reactive antilymphocyte antibody, determined by both C-dependent cytotoxicity and indirect immunofluorescence. The antibody exhibited specificities for both autologous lymphocytes and lymphocytes from normal donors; cytotoxic activity for autologous leukaemia cells was removed by absorption with normal isologous tonsil lymphocytes. Specific enrichment of this antibody relative to the serum level was demonstrated in the cryoglobulin and its isolated 19S fractions. Free lymphocyte surface antigen was also demonstrated by gel diffusion using specific rabbit antilymphocyte antiserum. These data strongly suggest the presence of pathogenetically significant circulating complexes of lymphocyte surface antigen and specific antibody in certain patients with CLL.
D000799:Angioedema / Q000150:complications; D000906:Antibodies; D000936:Antigen-Antibody Complex; D000941:Antigens; D002462:Cell Membrane / Q000276:immunology; D003165:Complement System Proteins / Q000172:deficiency*; D003450:Cryoglobulins / Q000032:analysis; D003601:Cytotoxicity Tests, Immunologic; D006801:Humans; D007105:Immune Complex Diseases; D007075:Immunoglobulin M / Q000032:analysis; D007945:Leukemia, Lymphoid / Q000150:complications / Q000276:immunology*; D008214:Lymphocytes / Q000276:immunology*; D008297:Male; D008875:Middle Aged
D000906:Antibodies; D000936:Antigen-Antibody Complex; D000941:Antigens; D003450:Cryoglobulins; D007075:Immunoglobulin M; D003165:Complement System Proteins