Mechanisms of immune lysis of red blood cells in vitro. I. Paroxysmal nocturnal hemoglobinuria cells.
نویسندگان
چکیده
The effect of five different reactions which activate complement (antibody activation, reduction in ionic strength, acidification, cobra venom factor (CoF) activation, and inulin activation) upon normal and PNH cells was investigated, using normal serum and serum devoid of the fourth component of complement (C4) activity from patients with hereditary angioneurotic edema (HANE) as a source of complement. Both normal and HANE serum lysed paroxysmal nocturnal hemoglobinuria (PNH) cells when complement was activated by acidification, CoF and inulin, indicating that the terminal steps of complement were activated in the absence of C4, presumably by the alternate or properdin pathway. Normal but not HANE serum lysed cells coated with anti-I, indicating that complement was activated by the C1-dependent classic pathway. HANE serum partially supported lysis by serum at reduced ionic strength, indicating that the activation of terminal complement components had occurred through both of the pathways of activation. The amount of the third component of human complement (C3) which was bound to the membrane of lysed and unlysed cells by these procedures was determined by anti-C3 absorption and was found to differ for each method of complement activation. In general, more C3 was bound to lysed cells than to unlysed cells. For given conditions, more was bound to PNH cells than to normal cells. However, very much less bound C3 was required for lysis of the PNH cells than for lysis of normal cells. These two phenomena, especially the latter, account for the marked lysis of PNH cells when complement is activated. Normal cells treated with AET (aminoethylisothiouronium bromide) did not bind more C3 than untreated cells and the lysed cells had less bound C3 than lysed PNH cells.
منابع مشابه
Immune lysis of normal human and paroxysmal nocturnal hemoglobinuria (PNH) red blood cells. I. The sensitivity of PNH red cells to lysis by complement and specific antibody.
متن کامل
Leukocyte antigen-antibody reaction and lysis of paroxysmal nocturnal hemoglobinuria erythrocytes.
T HE TRANSFUSION OF COMPATIBLE WHOLE BLOOD to patients suffering from paroxysmal nocturnal hemoglobinuria ( PNH ) is known to sometimes cause a hemolytic reaction. It has been suggested that leukocyte isoantibodies present in the patient’s serum through interaction with the donor’s leukocyte antigens are responsible for lysis of PNH red cells.’ As yet, it has not been clarified why this interac...
متن کاملSerum Anti-YG Globulin Factors in Paroxysmal Nocturnal Hemoglobinuria
I N PAROXYSMAL NOCTURNAL HEMOGLOBINURIA (PNH) accelerated destruction of abnormal red cells occurs within an apparently benign extracorpuscular environment.1 Although the underlying cellular defect has not yet been fully elucidated, it has long been recognized that, in vitro, PNII erythrocytes are extraordinarily susceptible to immune lysis. The possibility exists that, in vivo, destruction of ...
متن کاملMechanisms of immune lysis of the red cells in hereditary erythroblastic multinuclearity with a positive acidified serum test and paroxysmal nocturnal hemoglobinuria.
The red cells of patients with hereditary erythroblastic multinuclearity with a positive acidified serum test (HEMPAS), a form of congenital dyserythropoietic anemia, and the cells of patients with paroxysmal nocturnal hemoglobinuria (PNH) are lysed more readily than normal cells by certain antibodies, notably cold agglutinins (anti-I) and complement. With some but not other examples of anti-I,...
متن کاملNormal human lymphocytes treated in vitro with the sulfhydryl compound AET: relationship to the lymphocytes of paroxysmal nocturnal hemoglobinuria.
Because in vitro treatment with the sulfhydrsl compound AET is known to alter normal red cells in such a way that they become highly susceptible to the lytic action of complement, an investigation was carried out to evaluate whether the same substance could modify normal lymphocytes in a similar way. The lymphocytes from paroxysmal nocturnal hemoglobinuria were also studied as a comparison. AET...
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ورودعنوان ژورنال:
- The Journal of clinical investigation
دوره 52 5 شماره
صفحات -
تاریخ انتشار 1973