RESUMO
【Objective】 To evaluate the coagulation function of children with Kasabach-Merritt syndrome(KMS)by thromboelastography (TEG) and conventional coagulation tests (CCTs), and to explore the correlation and consistency of the 2 test methods. 【Methods】 A total of 49 children with KMS, submitted to our hospital from January 2016 to December 2020, were enrolled. The TEG, CCTs data and platelet count were analyzed to evaluate the coagulation function, and the superiority of the 2 test methods were compared by Spearman correlation and Kappa consistency analysis. 【Results】 TEG and CCTs showed that the coagulation reaction time(R) was normal, the counts and function of platelet and fibrinogen decreased, and the D-dimer increased. The coagulation complex index (CI) indicated that the whole coagulation function was low. There was no significant difference in coagulation by sex or age in KMS children. The correlation analysis of TEG and CCTs in the coagulation function of KMS children showed that R was correlated with prothrombin time (PT) and activated partial thromboplastin Time(APTT), respectively (P<0.01); Fib had weak correlation with clot formation time (k)(r2=0.33), but strongly correlated with α-angle and MA value(r2=0.7, 0.69), respectively (P<0.01). PLT was moderately correlated with MA(r2=0.49, P<0.05); D-dimer had no correlation with LY30. Comparision resu lts of the consistency of TEG and CCTs showed that FIB and MA had consistency ( kappa=1, P<0.01); None or weak consistency was noticed among other indicators, R with PT/APTT, the kappa was 0.18 and 0.19; Fib with K/α-Angle, the kappa was 0.28 and 0.34; D-dimer with LY30, the kappa was 0.01; PLT with MA, the kappa was 0.35. 【Conclusion】 The main manifestations in low coagulation function in children with KMS were mainly thrombocytopenia, lower fibrinogen, and increased fibrinogen degradation-products, and the coagulation factors were normal. Except for Fib and MA, the consistency of other indexes in the detection of coagulation function in children with KMS by TEG and CCT is weak. Some indexes are significantly correlated but others not. Therefore, the 2 test methods are irreplaceable and should be combined to reduce the risk of embolism and bleeding in children.