Thromboelastographic and hemostatic characteristics in pediatric patients with sickle cell disease.
BACKGROUND
Patients with sickle cell disease suffer from a variety of vaso-occlusive events that may be related to activation of the hemostatic system. Thromboelastography assesses the functionality of this system from a global standpoint and has demonstrated some utility in detecting hypercoagulable states in varied clinical settings, but it has not been systematically evaluated in patients with sickle cell disease.


OBJECTIVE
To characterize the findings of thromboelastography in patients with sickle cell disease during periods of steady state and illness, to compare these results with those of healthy controls, and to correlate these profiles with other measured hemostatic parameters.


METHODS
In this cross-sectional study, we obtained thromboelastographic and other hemostatic data on specimens from 46 patients with sickle cell disease (35 with hemoglobin SS, 7 with hemoglobin SC, and 4 with hemoglobin S-beta thalassemia) and 20 healthy race-matched controls. Data were obtained from patients with sickle cell disease at baseline conditions (n = 41) and in the setting of acute illness (n = 5).


RESULTS
Patients with hemoglobin SS had lower reaction time and higher angle, maximum amplitude, and coagulation index values on thromboelastography than the control group. Hemoglobin SC patients had higher angle, maximum amplitude, and coagulation index values than controls. Hemoglobin S-beta thalassemia patients showed no significant differences compared with controls. Five hemoglobin SS patients with recent or current illness demonstrated increased maximum amplitude and coagulation index compared with hemoglobin SS patients at baseline conditions.


CONCLUSIONS
Patients with sickle cell disease demonstrated a significant hypercoagulable state in thromboelastography profiles, with the degree of abnormality dependent on the type of sickle cell disease and perhaps the presence of acute illness. Continued follow-up of this patient cohort, as well as further study of larger and more homogeneous patient groups, is required to adequately assess the utility of thromboelastography in predicting complications of sickle cell disease.
Yee
Donald L
DL
Department of Pediatrics, Cancer Center & Hematology Service, Baylor College of Medicine, Houston, Tex 77030, USA.
Edwards
Rachel M
RM
Mueller
Brigitta U
BU
Teruya
Jun
J
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000473:pathology*; D001777:Blood Coagulation; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D004906:Erythrocyte Count; D004909:Erythrocyte Indices; D004913:Erythrocytes, Abnormal; D005260:Female; D006455:Hemoglobins, Abnormal / Q000032:analysis; D006801:Humans; D007223:Infant; D008297:Male; D013916:Thrombelastography / Q000379:methods*
D006455:Hemoglobins, Abnormal