Hemostatic analysis of a 13 year old with antiphospholipid syndrome and restrictive pericarditis.
Antiphospholipid syndrome is an autoimmune thrombophilic disorder that is uncommon in adults and remarkably rare in children. Thrombotic etiological factors are variable in antiphospholipid syndrome, including antibody-antigen complex-mediated platelet activation, inhibition of anticoagulants, or attenuation of fibrinolysis. We present the case of a child with antiphospholipid syndrome presenting with syncope, constrictive pericarditis and hepatic enlargement that was found to have platelet-mediated hypercoagulability and marked clot lysis via thrombelastography in the preoperative period. Restoration of circulation following pericardectomy and inotropic support was associated with attenuation of hypercoagulability and fibrinolysis. It is concluded that the etiological factors responsible for antiphospholipid syndrome-mediated hemostatic abnormalities and the probable effects of hepatic hypoperfusion on clot lysis in this patient were detected with thrombelastography, and similar thrombelastographic analyses are recommended to compliment standard coagulation assessments of patients with antiphospholipid syndrome.
Nielsen
Vance G
VG
Department of Anesthesiology, The University of Alabama at Birmingham, Birmingham, Alabama 35294-2172, USA. vnielsen@uab.edu
Steenwyk
Brad L
BL
Burch
Thomas M
TM
King
Colin K
CK
McGiffin
David C
DC
D000293:Adolescent; D000936:Antigen-Antibody Complex / Q000276:immunology*; D016736:Antiphospholipid Syndrome / Q000097:blood / Q000150:complications*; D001778:Blood Coagulation Disorders / Q000175:diagnosis* / Q000276:immunology*; D005342:Fibrinolysis / Q000276:immunology; D006487:Hemostasis / Q000276:immunology; D048550:Hepatic Insufficiency / Q000276:immunology; D006801:Humans; D008102:Liver Circulation / Q000276:immunology; D008297:Male; D010492:Pericardiectomy; D010494:Pericarditis, Constrictive / Q000150:complications* / Q000601:surgery; D015424:Reperfusion; D013916:Thrombelastography
D000936:Antigen-Antibody Complex