T-lymphocytes escape membrane defect in paroxysmal nocturnal haemoglobinuria.
Erythrocytes, granulocytes and platelets from patients with paroxysmal nocturnal haemoglobinuria (PNH) are abnormally sensitive to lysis by complement. We studied T-lymphocytes from PNH patients for abnormal complement lysis sensitivity. T-lymphocytes free of other contaminating blood cells were prepared by sedimentation, nylon wool filtration, and density gradient centrifugation. The lymphocytes were then labelled with 51Cr and lysis induced by antithymocyte globulin and rabbit complement. PNH lymphocytes were no more susceptible to complement-mediated lysis than lymphocytes from normal individuals. The unusual sensitivity of PNH erythrocytes could still be demonstrated when rabbit serum was a source of complement so the lack of any difference in the sensitivity of normal and PNH lymphocytes was probably not attributable to the inability of rabbit serum to elicit the membrane defect. PNH erythrocytes and granulocytes also acquire more membrane-bound C3 when human complement is activated. Therefore we also searched for increased membrane C3 binding on PNH lymphocytes using anti-I antibody and human serum as a complement source. C3 binding was measured using 125I labelled monoclonal mouse anti-human C3. While we verified increased membrane C3 binding on PNH granulocytes during complement activation we were unable to show similar differences between PNH and normal T-lymphocytes. Thus PNH T-lymphocytes do not share the membrane abnormalities of PNH-erythrocytes and granulocytes.
D000368:Aged; D000818:Animals; D002462:Cell Membrane / Q000378:metabolism; D003167:Complement Activation; D003176:Complement C3 / Q000378:metabolism; D003168:Complement Fixation Tests; D003165:Complement System Proteins / Q000378:metabolism*; D004910:Erythrocyte Membrane / Q000378:metabolism; D005260:Female; D006098:Granulocytes / Q000378:metabolism; D006457:Hemoglobinuria, Paroxysmal / Q000097:blood*; D006461:Hemolysis; D006801:Humans; D066298:In Vitro Techniques; D008297:Male; D008875:Middle Aged; D011817:Rabbits; D013601:T-Lymphocytes / Q000378:metabolism*
D003176:Complement C3; D003165:Complement System Proteins