Catastrophic antiphospholipid syndrome in lupus-associated immune thrombocytopenia treated with eltrombopag A case series and literature review

Abstract

Background: Eltrombopag, a thrombopoietin receptor (TPO-R) agonist, is considered a second-line treatment for patients with refractory immune thrombocytopenia (ITP). Systemic lupus erythematosus (SLE) is frequently associated with ITP. In some cases, thrombocytopenia in SLE patients is attributed to concurrent antiphospholipid antibodies (APLA). Currently, data regarding treatment with TPO-R agonists for ITP in SLE or APLA patients are limited. The incidence of SLE flare or antiphospholipid syndrome while on TPO-R agonists has not been well-studied. Cases: We report 2 cases of female patients with SLE and concurrent triple positive APLA, without thrombotic events in their medical history, in our rheumatology clinic, who were treated for refractory ITP with eltrombopag. Both developed catastrophic antiphospholipid syndrome a few weeks after beginning treatment with eltrombopag. They were admitted to the intensive care unit and treated with solumedrol, plasmapheresis, anticoagulation and rituximab. Conclusions: We describe a severe possible side-effect of eltrombopag as a trigger of catastrophic antiphospholipid syndrome, a rare initial manifestation of antiphospholipid syndrome, in SLE patients with APLA. We suggest that APLA should be tested before initiating eltrombopag in patients with SLE-associated ITP. The safety of this treatment should be considered in these cases.

Antiphospholipid antibodies as potential predictors of disease severity and poor prognosis in systemic lupus erythematosus-associated thrombocytopenia: results from a real-world CSTAR cohort study

“…The pathophysiological mechanisms underlying thrombocytopenia in SLE patients are not fully known, but at least three mechanisms have been identified: impaired production of platelets in the bone marrow, sequestration of platelets in the spleen, or accelerated destruction of platelets in the peripheral circulation [ 12 ]. aPLs may play an important role in these processes [ 13 , 14 ].While it is not officially classified as a criterion, a reduced platelet count is a frequently observed laboratory characteristic in patients with anti-phospholipid syndrome (APS), regardless of whether they have a concurrent diagnosis of SLE. aPLs, which can appear in SLE and APS patients, are a heterogeneous group of autoantibodies reacting against phospholipids, phospholipid-protein complexes, and phospholipid-binding proteins, including lupus anticoagulant (LA), anticardiolipin (aCL) and anti-beta2 glycoprotein I (anti-β2GP1) antibodies [ 15 ].…”

Antiphospholipid Antibodies as Potential Predictors of Disease Severity and Poor Prognosis in Systemic Lupus Erythematosus-Associated Thrombocytopenia: Results from a Real-World CSTAR Cohort Study

“…The pathophysiological mechanisms underlying thrombocytopenia in SLE patients are not fully known, but at least three mechanisms have been identi ed: impaired production of platelets in the bone marrow, sequestration of platelets in the spleen, or accelerated destruction of platelets in the peripheral circulation [12] . aPLs may play an important role in these processes [13,14] . Antiphospholipid antibodies, which can appear in SLE and anti-phospholipid syndrome (APS) patients, are a heterogeneous group of autoantibodies reacting against phospholipids, phospholipid-protein complexes, and phospholipid-binding proteins, including LA, ACL and anti-β2GP1 antibodies [15] .…”

Catastrophic Antiphospholipid Syndrome

“…Further research is required to determine the occurrence of CAPS after taking eltrombopag. However, the authors of the article recommend testing for APLA before prescribing eltrombopag to patients with ITP associated with SLE [ 88 ].…”