Abstract
Severe thrombooytopenia, characterized by small platelets, is a major cause of morbidity and mortality in the Wiskott-Aldrich Syndrome (WAS). This microthrombocytopenia was assumed to reflect a primary defect in platelet production until the recent demonstration that splenectomy (Spx) frequently normalizes the platelet count. We have measured platelet mean volume (PMV) and platelet associated immunoglobulin (PAIgG) in WAS patients before and after Spx. WAS patients (n=14) average MPV pre-Spx was 3.2 μ3 ± .2 s.e.m. compared to normal controls' MPV of 6.6 μ3 ± .6, p,.<001. 7 WAS patients underwent Spx, in each case a normal platelet count was achieved and the group MPV rose to 5.8 μ3 ± .3, within the normal range. PAIgG per platelet in the WAS subjects (n=10) pre-Spx ranged from 18.1 fg to 267.3 fg mean 78.9 fg ± 23.3, significantly greater than normal (<4 fg/platelet) and aplastic controls, p<.001. In 5 WAS subjects studied post-Spx, PAIgG mean declined to normal, ranging from 1.6 fg to 8.7 fg, with a mean 4.0 fg ± 1.25. Increase in MPV and decrease in PAIgG usually occurred within two weeks of Spx. Based upon the changes in MPV and PAIgG, we speculate that immune mediated platelet destruction occurs in WAS subjects, that this process requires an intact spleen and a complex interaction between the WAS IgG-coated platelet and splenic macrophages resulting in piecemeal loss of platelet membrane giving rise to the small WAS platelet.
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Corash, L., Shafer, B., Rosse, W. et al. 804 PLATELET ASSOCIATED IMMUNOGLOBULIN, PLATELET SIZE AND THE EFFECT OF SPLENECTOMY IN THE WISKOTT-ALDRICH SYNDROME. Pediatr Res 15 (Suppl 4), 576 (1981). https://doi.org/10.1203/00006450-198104001-00828
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DOI: https://doi.org/10.1203/00006450-198104001-00828