Abstract

ABSTRACTObjectives: The relevance of detecting antibodies against anticardiolipin, β2-glycoprotein I (β2gpI) or lupus anticoagulant (LA), collectively called antiphospholipid autoantibodies (APA), in subjects with immune thrombocytopenia (ITP) is still a debated issue. In particular, whether APA profile may affect the clinical course of ITP is unknown.Methods: In this study, we report our experience in a cohort of ITP patients with APA with specific interest to the relevance of different antiphospholipid antibody profiles in clinical outcome and response to treatment.Results: Thirty-seven out of 159 patients (23.2%) fulfilling ITP criteria had a platelet count ≤50 × 109/L and tested positive at APA at ITP onset. Twenty-three (62.1%) patients received at least one line of treatment for ITP. Fourteen subjects (37.8%) showing triple positivity for APA showed a significantly lower median platelet count compared to other APA patients (p = .006). Among these ITP subjects with triple positivity, 85.7% needed a treatment because of low platelet count compared to 47.8% ITP patients with non-triple-positive APA (p = .0094). ITP/APA subjects who received immunosuppressors had a higher rate of thrombosis (p = .024) as well as thrombosis developed in subjects who were on steroid therapy at a significantly higher dosage than subjects who did not develop thrombotic episodes (p < .001). When considering treatment, CR and SR rate were significantly higher in ITP/triple-positive patients compared to non-triple-positive subjects (p = .021 and p = .005).Conclusions: The profile of APA may affect the outcome of patients with ITP.

Highlights

  • Immune thrombocytopenia (ITP) comprises a heterogeneous group of disorders characterized by autoimmune-mediated platelet destruction and impairment of platelet production leading to bleeding events [1,2]

  • In this study, we report our experience in a cohort of immune thrombocytopenia (ITP) patients with antiphospholipid autoantibodies (APA) with specific interest to the relevance of different antiphospholipid antibody profiles in clinical outcome and response to treatment

  • It seems advisable to test for APA any ITP patient developing thrombosis, it is still unclear if patients with primary ITP and APA should be treated differently

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Summary

Introduction

Immune thrombocytopenia (ITP) comprises a heterogeneous group of disorders characterized by autoimmune-mediated platelet destruction and impairment of platelet production leading to bleeding events [1,2]. Antiphospholipid syndrome (APS) is characterized by arterial and venous thrombosis, recurrent foetal loss, and thrombocytopenia in the presence of antiphospholipid antibodies (APAs). APAs can coexist in subjects with ITP and typically, platelet count is lower than in isolated APAs. The percentage of ITP patients positive for APA varies from 25 to 75% [3,4,5]. It has been previously reported that APA positivity is a common finding in patients with ITP. The most addressed aspect of this association has been the increased risk of thrombosis and this implication seems the only reason for testing ITP subjects for APA. Previous studies failed to identify clinical features which may differentiate ITP from ITP with APA

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