Bleeding in Patients with Antiphospholipid Antibodies

Abstract

The antiphospholipid antibodies (aPL) are commonly associated with thrombotic events and obstetric complications. However, apart from the bleeding complications of antithrombotic therapy, the acquired coagulopathy caused by the aPL, particularly by lupus anticoagulant and anticardiolipin antibodies, might be occasionally manifested as a hemorrhagic syndrome with various clinical severity. Bleeding symptoms vary from mild (mucocutaneous) up to life-threatening (gastrointestinal, intracranial). The bleeding may be the first manifestation of aPL or appear concomitantly with thrombosis. The underlying hemostatic changes include thrombocytopenia, platelet function disorders, and coagulation factor inhibitors or deficiencies, namely prothrombin, FVII, FVIII, FX, and FXI. Thrombocytopenia is the most common finding, seen in up to 53% of patients with aPL, although it is usually mild to moderate and associated with significant bleeding only in a minority of cases. Of interest, patients with severe thrombocytopenia appear to be less likely to suffer from thrombotic events. The involved pathophysiological mechanisms are heterogeneous. Non-neutralizing antibodies against coagulation factors resulting in increased clearance, specific antibodies against platelet membrane glycoproteins, increasing platelet activation and aggregation with subsequent consumption, and immune-mediated platelet clearance are among those identified. Immunosuppression, preferably with corticosteroids, represents the first-choice therapeutic approach. Plasmapheresis is efficient in the case of catastrophic antiphospholipid syndrome. Antithrombotic therapy can be challenging, but its administration should continue as much as possible.

A mysterious hematochezia in an adolescent boy: atypical presentation of childhood systemic lupus erythematosus

“…3 Monotherapy with corticosteroid is efficient in most cases. 5 In our case, he is being treated with oral prednisolone along with hydroxychloroquine and methotrexate. He showed response in form of normalisation of PT with no further bleeding episode during follow-up, implying resolution of prothrombin deficiency state.…”

“…4 Acquired deficiency of factor-II is the most well-known among the all coagulation factor deficiencies in presence of anti-phospholipid antibodies and this condition is known as lupus anticoagulant hypoprothombinemia syndrome (LAHS). 5 This deficiency of factor-II is attributed to the presence of antibody against the prothrombin which leads to bleeding manifestations.…”

“…Since the risk of thrombosis is still there due to presence of antiphospholipid antibodies, both the risk for bleeding and thrombosis should be carefully evaluated during treatment and follow-up and if required the bleeding cessation therapy should be counterweighted by antithrombotic therapy. 5…”