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Platelet-associated immunoglobulin, platelet size, and the effect of
splenectomy in the Wiskott-Aldrich syndrome
L Corash, B Shafer and RM Blaese
Wiskott-Aldrich syndrome (WAS) thrombocytopenia is frequently improved by
splenectomy, although the mechanism of the thrombocytopenia and its
resolution are unknown. Previous studies in two patients have shown that
mean platelet volume, which is characteristically reduced in WAS, increased
along with platelet count postsplenectomy. Additional studies in a limited
number of patients have also demonstrated that platelet- associated
immunoglobulin G (PAIgG) is elevated presplenectomy, but to date no
postsplenectomy data have been reported. The present study was performed to
more fully evaluate the effect of splenectomy on platelet volume and PAIgG
in WAS. Before splenectomy, mean platelet volume was reduced but platelet
size was broadly distributed with substantial overlap of the normal range.
PAIgG was significantly elevated in 13 of 14 presplenectomy WAS patients
(means = 78.9 fg per platelet) and fell to normal levels postoperatively
(means = 4.0 fg per platelet). Platelet count and clinical status improved
postsplenectomy, and mean platelet volume and platelet volume distribution
returned to the normal range. WAS subjects who relapsed with recurrent
thrombocytopenia redeveloped elevated PAIgG but maintained normal platelet
size. The spleen appears to play a critical role in a process that may be
immunologically mediated and results in reduced platelet size.
Volume 65,
Issue 6,
pp. 1439-1443,
06/01/1985
Copyright © 1985 by The American Society of Hematology
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