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How I treat catastrophic thrombotic syndromes.

Publication ,  Journal Article
Ortel, TL; Erkan, D; Kitchens, CS
Published in: Blood
September 10, 2015

Catastrophic thrombotic syndromes are characterized by rapid onset of multiple thromboembolic occlusions affecting diverse vascular beds. Patients may have multiple events on presentation, or develop them rapidly over days to weeks. Several disorders can present with this extreme clinical phenotype, including catastrophic antiphospholipid syndrome (APS), atypical presentations of thrombotic thrombocytopenic purpura (TTP) or heparin-induced thrombocytopenia (HIT), and Trousseau syndrome, but some patients present with multiple thrombotic events in the absence of associated prothrombotic disorders. Diagnostic workup must rapidly determine which, if any, of these syndromes are present because therapeutic management is driven by the underlying disorder. With the exception of atypical presentations of TTP, which are treated with plasma exchange, anticoagulation is the most important therapeutic intervention in these patients. Effective anticoagulation may require laboratory confirmation with anti-factor Xa levels in patients treated with heparin, especially if the baseline (pretreatment) activated partial thromboplastin time is prolonged. Patients with catastrophic APS also benefit from immunosuppressive therapy and/or plasma exchange, whereas patients with HIT need an alternative anticoagulant to replace heparin. Progressive thrombotic events despite therapeutic anticoagulation may necessitate an alternative therapeutic strategy. If the thrombotic process can be controlled, these patients can recover, but indefinite anticoagulant therapy may be appropriate to prevent recurrent events.

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Published In

Blood

DOI

EISSN

1528-0020

Publication Date

September 10, 2015

Volume

126

Issue

11

Start / End Page

1285 / 1293

Location

United States

Related Subject Headings

  • Thrombosis
  • Thrombocytopenia
  • Syndrome
  • Purpura, Thrombotic Thrombocytopenic
  • Pregnancy
  • Plasma Exchange
  • Male
  • Immunology
  • Humans
  • Heparin
 

Citation

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Chicago
ICMJE
MLA
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Ortel, T. L., Erkan, D., & Kitchens, C. S. (2015). How I treat catastrophic thrombotic syndromes. Blood, 126(11), 1285–1293. https://doi.org/10.1182/blood-2014-09-551978
Ortel, Thomas L., Doruk Erkan, and Craig S. Kitchens. “How I treat catastrophic thrombotic syndromes.Blood 126, no. 11 (September 10, 2015): 1285–93. https://doi.org/10.1182/blood-2014-09-551978.
Ortel TL, Erkan D, Kitchens CS. How I treat catastrophic thrombotic syndromes. Blood. 2015 Sep 10;126(11):1285–93.
Ortel, Thomas L., et al. “How I treat catastrophic thrombotic syndromes.Blood, vol. 126, no. 11, Sept. 2015, pp. 1285–93. Pubmed, doi:10.1182/blood-2014-09-551978.
Ortel TL, Erkan D, Kitchens CS. How I treat catastrophic thrombotic syndromes. Blood. 2015 Sep 10;126(11):1285–1293.

Published In

Blood

DOI

EISSN

1528-0020

Publication Date

September 10, 2015

Volume

126

Issue

11

Start / End Page

1285 / 1293

Location

United States

Related Subject Headings

  • Thrombosis
  • Thrombocytopenia
  • Syndrome
  • Purpura, Thrombotic Thrombocytopenic
  • Pregnancy
  • Plasma Exchange
  • Male
  • Immunology
  • Humans
  • Heparin