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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.
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| Copyright © 1979 by American Society of Hematology Online ISSN: 1528-0020 | |||||||||