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Two populations of granulocytes in paroxysmal nocturnal hemoglobinuria

M Stern and WF Rosse

The granulocytes in paroxysmal nocturnal hemoglobinuria (PNH) are defective, and the defect is similar to that previously described for the PNH erythrocyte. Using anti-I antibody to activate complement and 51Cr release to detect cell lysis, we found two populations of granulocytes that differed in their susceptibility to lysis by complement in 5 of 6 patients. A proportion of the cells were lysed by one-fifteenth to one-twentieth the amount of complement required to lyse normal cells; the remainder of the granulocytes appeared to be normal in their susceptibility to the lytic action of complement. The binding of the third component of complement (C3) to PNH granulocytes was at least twice that bound to normal cells, even though the binding of antibody was the same for normal and PNH cells. This suggests that the binding of C3 and probably the efficiency of the terminal steps of complement lysis are increased in the abnormal PHN granulocyte. These defects affect only a portion of the granulocytes, thus suggesting that the disorder is a clonal stem cell abnormality.

Volume 53, Issue 5, pp. 928-934, 05/01/1979
Copyright © 1979 by The American Society of Hematology


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T. Shichishima, Y. Saitoh, T. Terasawa, K. Ogawa, and Y. Maruyama
Relationship Between the Phenotypes of Circulating Erythrocytes and Cultured Erythroblasts in Paroxysmal Nocturnal Hemoglobinuria
Blood, July 1, 1997; 90(1): 435 - 443.
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  Copyright © 1979 by American Society of Hematology         Online ISSN: 1528-0020