How I treat catastrophic thrombotic syndromes

Abstract

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.

Idiopathic catastrophic thrombosis with happy ending

“…Since there was no evidence of other causes of catastrophic thrombotic syndromes such as catastrophic antiphospholipid syndrome, atypical thrombotic thrombocytopenic purpura or cancer-associated thrombosis, we are certain that another unidentified strong prothrombotic state (‘idiopathic’ catastrophic thrombotic syndrome)7 was the cause for the extreme clinical presentation of our patient. Except for increased factor VIII levels which could be explained by acute phase reaction, we have not identified any further thrombophilic factor.…”

“…We have not initiated additional therapies such as plasmapheresis and/or immunomodulatory agents as indicated in catastrophic antiphospholipid syndrome or atypical thrombotic thrombocytopenic purpura 7. If HIT could have been ruled out, UFH would also have been an appropriate anticoagulant in this condition.…”

Acute syndrome of pan-epidermolysis and thrombotic storm arising in a patient with systemic lupus erythematosus

“… 7 Her development of multiple thrombotic events including renal infarctions, ischemic leg requiring amputation, deep venous thrombosis, and microvascular thrombi on biopsy were consistent with a thrombotic storm. 8 In the reported cases of TEN-like SLE, corticosteroids, IVIg and wound care are described as the cornerstones of treatment. 2 Most of these reported patients survive and enter remission.…”

Das Antiphospholipid-Syndrom – Eine interdisziplinäre Herausforderung

“…Als Ursache wird ein "Thrombin-Sturm" verantwortlich gemacht. Differenzialdiagnostisch ist die Erkrankung schwer von anderen mikroangiopathischen Erkrankungen, wie einer thrombotisch-thrombozytopenischen Purpura (TTP) oder einem hämolytischurämischen Syndrom (HUS), abzugrenzen [26].…”