Abstract:

Objective To compare the difference between the maximum amplitude（MA） values of thromboelastography（TEG） and maximum aggregation rate（MAR） of different agglomerators in Glanzmann thrombasthenia（GT） and Bernard-Soulier syndrome（BSS）. Methods Totally，from July 2014 to June 2021，24 GT and 13 BSS patients were enrolled from Beijing Children’s Hospital of Capital Medical University，and another 20 patients with coagulation factor deficiency were enrolled as control group. Light transmittance aggregometry（LTA） and TEG were used to compare the differences in platelet aggregation function among the groups. Results The MA value and MAR induced by adenosine diphosphate（ADP），collagen（Col） and arachidonic acid（AA） of GT group were lower than those of BSS group and control group（P<0.01）. Ristocetin（Risto） induced MAR in GT group was lower than that in control group（P<0.01）. Risto induced MAR in BSS group was lower than those in GT group and control group（P<0.01）. Spearman correlation analysis showed that there was no correlation between MAR and MA values induced by the 4 agglomerators in GT，BSS and control group（P>0.05）. Conclusions The combined determination of MAR with multiple agglomerators can reflect the platelet function of children with congenital bleeding disorders better. Therefore，MAR is more sensitive than MA value in diagnosing and distinguishing GT and BSS. MA value can not be a diagnostic marker of BSS.

Key words: Maximum aggregation rate, Thromboelastography, Glanzmann thrombasthenia, Bernard-Soulier syndrome