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

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.

The challenges in diagnosing pediatric primary antiphospholipid syndrome

“…3 Both in adult and pediatric APS series, thrombocytopenia is the most frequently reported non-criteria manifestation. 13 One of our patients had presented with chorea before the thrombotic event and had valvular heart disease, as well. Chorea is a hyperkinetic movement disorder that develops due to basal ganglia damage.…”

“…One patient did not have any additional prothrombotic risk factors. The median age at symptom onset was 16 (10)(11)(12)(13)(14)(15)(16)(17) years. The median (min-max) follow-up time (from the time of APS diagnosis to the last visit) was 32 (6-77) months.…”

Detection of IgA Antiphospholipid Antibodies Does not Improve Thrombotic Antiphospholipid Syndrome Classification: A two-Center Study

“…Expression of platelet membrane glycoproteins increases after aPL stimulation, and the binding of aβ 2 GPI-β 2 GPI complex to receptors on the platelet membrane induces the activation and aggregation of platelets. 33 In summary, aPLs (including IgA isotype) may promote platelet aggregation; thrombocytopenia may be a consequence of platelet consumption. Similar to Vlagea et al, 11 Bertolaccini et al, 34 and others, we failed to find any association between thrombosis and IgA aPL.…”

Immune Thrombocytopenic Purpura as a Hemorrhagic Versus Thrombotic Disease: An Updated Insight into Pathophysiological Mechanisms

“…Some publications have shown the coexistence of APA and ITP [115,116]. According to the current guidelines, when APA are detected in an ITP patient without a history of thrombosis or obstetric complications, this finding will not change the diagnosis of primary ITP [117,118].…”