Journal
LUPUS
Volume 28, Issue 14, Pages 1663-1668Publisher
SAGE PUBLICATIONS LTD
DOI: 10.1177/0961203319886915
Keywords
Beta2-glycoprotein I; antiphospholipid syndrome; catastrophic illness; platelet function tests; microcirculation
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Platelet activation and decrease in platelet count characterize the development of the most feared form of antiphospholipid syndrome (APS), i.e. catastrophic APS (CAPS). We aimed to assess if immuno-affinity purified anti-beta 2-glycoprotein I (a beta 2GPI) antibodies enhance platelet activation inducing a significant flow obstruction in a platelet function analyzer (PFA). Affinity purified a beta 2GPI antibodies were obtained from 13 triple positive patients with a strong lupus anticoagulant (LA) and high titers of IgG anticardiolipin antibodies (aCL) and IgG a beta 2GPI. Platelet activation stimulated by adenosine diphosphate (ADP) in the presence or absence of a beta 2GPI was measured by the expression of P-selectin on platelet surface using flow cytometry. P-selectin expression remained close to baseline when normal whole blood was incubated with a beta 2GPI alone. When stimulated using a beta 2GPI combined with ADP, P-selectin expression (28.42 +/- 5.15% vs. 20.98 +/- 3.94%, p = 0.0076) was significantly higher than ADP alone. Closure time of normal whole blood passed through the PFA was significantly shorter using affinity purified a beta 2GPI than control IgG both in Col/ADP (160.1 +/- 62.1 s vs. 218.6 +/- 43.8 s; p = 0.021) and Col/EPI cartridges (149.5 +/- 26.7 s vs. 186.9 +/- 45.5 s; p = 0.030). Thus, platelet activation is enhanced by a beta 2GPI antibodies with a consequent premature closure in a PFA, possibly resembling that in microcirculation in patients with CAPS.
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