Hemostatic analysis of a 13 year old with antiphospholipid syndrome and restrictive pericarditis

Vance G Nielsen, Brad L. Steenwyk, Thomas M. Burch, Colin K. King, David C. McGiffin

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)695-697
Number of pages3
JournalBlood Coagulation and Fibrinolysis
Volume18
Issue number7
DOIs
StatePublished - Oct 2007
Externally publishedYes

Fingerprint

Antiphospholipid Syndrome
Pericarditis
Hemostatics
Thrombelastography
Thrombophilia
Fibrinolysis
Pericardiectomy
Constrictive Pericarditis
Preoperative Period
Liver
Platelet Activation
Syncope
Antigen-Antibody Complex
Anticoagulants
Blood Platelets

Keywords

  • Antiphospholipid syndrome
  • Fibrinolysis
  • Hypercoagulability
  • Pericarditis
  • Thrombelastography
  • Thrombosis

ASJC Scopus subject areas

  • Hematology

Cite this

Hemostatic analysis of a 13 year old with antiphospholipid syndrome and restrictive pericarditis. / Nielsen, Vance G; Steenwyk, Brad L.; Burch, Thomas M.; King, Colin K.; McGiffin, David C.

In: Blood Coagulation and Fibrinolysis, Vol. 18, No. 7, 10.2007, p. 695-697.

Research output: Contribution to journalArticle

Nielsen, Vance G ; Steenwyk, Brad L. ; Burch, Thomas M. ; King, Colin K. ; McGiffin, David C. / Hemostatic analysis of a 13 year old with antiphospholipid syndrome and restrictive pericarditis. In: Blood Coagulation and Fibrinolysis. 2007 ; Vol. 18, No. 7. pp. 695-697.
@article{48e80d3c850749f099af5745c8120677,
title = "Hemostatic analysis of a 13 year old with antiphospholipid syndrome and restrictive pericarditis",
abstract = "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.",
keywords = "Antiphospholipid syndrome, Fibrinolysis, Hypercoagulability, Pericarditis, Thrombelastography, Thrombosis",
author = "Nielsen, {Vance G} and Steenwyk, {Brad L.} and Burch, {Thomas M.} and King, {Colin K.} and McGiffin, {David C.}",
year = "2007",
month = "10",
doi = "10.1097/MBC.0b013e3282a14c99",
language = "English (US)",
volume = "18",
pages = "695--697",
journal = "Blood Coagulation and Fibrinolysis",
issn = "0957-5235",
publisher = "Lippincott Williams and Wilkins",
number = "7",

}

TY - JOUR

T1 - Hemostatic analysis of a 13 year old with antiphospholipid syndrome and restrictive pericarditis

AU - Nielsen, Vance G

AU - Steenwyk, Brad L.

AU - Burch, Thomas M.

AU - King, Colin K.

AU - McGiffin, David C.

PY - 2007/10

Y1 - 2007/10

N2 - 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.

AB - 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.

KW - Antiphospholipid syndrome

KW - Fibrinolysis

KW - Hypercoagulability

KW - Pericarditis

KW - Thrombelastography

KW - Thrombosis

UR - http://www.scopus.com/inward/record.url?scp=34748921654&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=34748921654&partnerID=8YFLogxK

U2 - 10.1097/MBC.0b013e3282a14c99

DO - 10.1097/MBC.0b013e3282a14c99

M3 - Article

VL - 18

SP - 695

EP - 697

JO - Blood Coagulation and Fibrinolysis

JF - Blood Coagulation and Fibrinolysis

SN - 0957-5235

IS - 7

ER -