Immune Thrombocytopenia in Antiphospholipid Syndrome: Is It Primary or Secondary?

Riccardo Tomasello, Giulio Giordano, Francesco Romano, Federica Vaccarino, Sergio Siragusa, Alessandro Lucchesi, Mariasanta Napolitano, Riccardo Tomasello, Giulio Giordano, Francesco Romano, Federica Vaccarino, Sergio Siragusa, Alessandro Lucchesi, Mariasanta Napolitano

Abstract

Antiphospholipid syndrome (APS) is frequently associated with thrombocytopenia, in most cases mild and in the absence of major bleedings. In some patients with a confirmed APS diagnosis, secondary immune thrombocytopenia (ITP) may lead to severe thrombocytopenia with consequent major bleeding. At the same time, the presence of antiphospholipid antibodies (aPL) in patients with a diagnosis of primary ITP has been reported in several studies, although with some specific characteristics especially related to the variety of antigenic targets. Even though it does not enter the APS defining criteria, thrombocytopenia should be regarded as a warning sign of a "high risk" APS and thus thoroughly evaluated. The presence of aPL in patients with ITP should be assessed as well to stratify the risk of paradoxical thrombosis. In detail, besides the high hemorrhagic risk in secondary thrombocytopenia, patients with a co-diagnosis of APS or only antibodies are also at risk of arterial and venous thrombosis. In this narrative review, we discuss the correlation between APS and ITP, the mechanisms behind the above-reported entities, in order to support clinicians to define the most appropriate treatment strategy in these patients, especially when anticoagulant or antiplatelet agents may be needed.

Keywords: antiphospholipid antibodies; antiphospholipid syndrome; immune thrombocytopenia; lupus anticoagulant; thrombocytopenia.

Conflict of interest statement

M.N. acted as a consultant for Bayer, BIOFVIIIx, NovoNordisk, Sobi, Amgen and received speaker fees from Kedrion, Octapharma, Baxalta, CSLBehring, Novo Nordisk, and Bayer. No potential conflict of interests has been declared by the other authors. S.S. acted as a consultant for Bayer, BIOFVIIIx, NovoNordisk, Sobi, Amgen, Novartis and received speaker Takeda, CSLBehring, Novo Nordisk, and Bayer. No potential conflict of interests has been declared by the other authors.

Figures

Figure 1
Figure 1
Algorithm for 1st and 2nd line treatment of SLE-associated thrombocytopenia. PDN: prednisone; MP: methylprednisolone; IVIG: intravenous immunoglobulins; HCQ: hydroxychloroquine; CS: corticosteroids; AZA: azathioprine; CSA: cyclosporine; CYC: cyclophosphamide; MMF: mycophenolate mofetil; d = days.

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