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Abnormal VIII: von Willebrand factor patterns in the plasma of patients
with the hemolytic-uremic syndrome
JL Moake, JJ Byrnes, JH Troll, CK Rudy, MJ Weinstein, NM Colannino and SL Hong
Plasma VIII:von Willebrand factor antigen (VIII:vWF) levels were elevated
approximately two- to eightfold in seven patients (three adults and four
children) during acute episodes of thrombocytopenia, renal failure, and
hemolytic anemia (the hemolytic-uremic syndrome, HUS). In all seven
patients, there was an alteration in plasma VIII:vWF patterns during these
acute HUS episodes, so that the largest VIII:vWF forms were relatively
decreased. Plasma VIII:vWF multimer patterns returned to normal, or nearly
to normal, as platelet counts returned to preexisting levels, even in the
patients whose recovery of renal function was incomplete and whose plasma
VIII:vWF antigen level remained above normal. The sister of one of the HUS
patients had a similar clinical prodrome (gastroenteritis) that was not
followed by thrombocytopenia or renal failure and was not accompanied by an
elevated level or abnormal forms of plasma VIII:vWF. These results suggest
that an alteration in VIII:vWF metabolism, distribution, or interaction
with platelets is associated with acute HUS episodes. In contrast to
patients with chronic relapsing thrombotic thrombocytopenic purpura, none
of the HUS patients (either during or after the acute HUS episodes) had a
defect in the conversion of unusually large VIII:vWF multimers derived from
endothelial cells to the VIII:vWF forms found in normal plasma.
Volume 64,
Issue 3,
pp. 592-598,
09/01/1984
Copyright © 1984 by The American Society of Hematology
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