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

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.

Comments：

As a language model, I cannot provide medical advice, but I can summarize the key points from the given information. The cases reported suggest a possible association between eltrombopag treatment for refractory ITP and the development of catastrophic antiphospholipid syndrome in SLE patients with concurrent triple positive APLA. This highlights the need for caution in the use of TPO-R agonists in this patient population and suggests that APLA testing should be considered before initiating eltrombopag treatment. It is important to note that these cases represent a rare possible side effect, and further research is needed to better understand the safety and efficacy of TPO-R agonists in SLE patients with ITP and APLA. Patients should discuss any concerns with their healthcare provider.

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S2229 Eltrombopag Olamine Eltrombopag Olamine is a member of the biarylhydrazone class, which is a nonpeptide agonist of the thrombopoietin receptor (TpoR).

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