Spontaneous heparin-induced thrombocytopenia syndrome: 2 new cases and a proposal for defining this disorder

The existence of spontaneous heparin-induced thrombocytopenia (HIT) syndrome (or autoimmune HIT), defined as a transient prothrombotic thrombocytopenic disorder without proximate heparin exposure serologically indistinguishable from HIT, is controversial. We describe 2 new cases presenting with thro...

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Veröffentlicht in:Blood 2014-06, Vol.123 (23), p.3651-3654
Hauptverfasser: Warkentin, Theodore E., Basciano, Paul A., Knopman, Jared, Bernstein, Richard A.
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Sprache:eng
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Zusammenfassung:The existence of spontaneous heparin-induced thrombocytopenia (HIT) syndrome (or autoimmune HIT), defined as a transient prothrombotic thrombocytopenic disorder without proximate heparin exposure serologically indistinguishable from HIT, is controversial. We describe 2 new cases presenting with thrombotic stroke/thrombocytopenia: one following shoulder hemi-arthroplasty (performed without heparin) and the other presenting to the emergency room without prior hospitalization, heparin exposure, or preceding infection. Both patients tested strongly positive for anti-platelet factor 4 (PF4)/heparin immunoglobulin (Ig)G in 2 different immunoassays and in the platelet serotonin-release assay. Crucially, both patients’ sera also caused strong (>80%) serotonin release in the absence of heparin, a serologic feature characteristic of delayed-onset HIT (ie, where heparin use precedes HIT but is not required for subsequent development or worsening of thrombocytopenia). We propose that a rigorous definition of spontaneous HIT syndrome should include otherwise unexplained thrombocytopenia/thrombosis without proximate heparin exposure and with anti-PF4/heparin IgG antibodies that cause strong in vitro platelet activation even in the absence of heparin. •Two well-documented cases of a HIT-mimicking disorder without proximate heparin exposure (spontaneous HIT syndrome) are reported.•The definition of spontaneous HIT syndrome should include strong serum-induced platelet activation at 0 IU/mL heparin (inhibited at 100 IU/mL).
ISSN:0006-4971
1528-0020
DOI:10.1182/blood-2014-01-549741