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Catastrophic antiphospholipid syndrome in an immune thrombocytopenia patient treated with avatrombopag

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DOI: 10.1016/j.rpth.2023.100125

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We report a case of a patient with chronic immune thrombocytopenia (ITP) who developed catastrophic antiphospholipid antibody syndrome (CAPS) following treatment with avatrombopag. The patient presented with symptoms including headache, nausea, and abdominal pain 3 weeks after initiation of avatrombopag, and diagnostic work-up revealed multiple microvascular thrombotic events. Laboratory tests showed positive antiphospholipid antibodies. The diagnosis of avatrombopag-associated CAPS was made.
Background: Avatrombopag is an orally administered second-generation thrombopoietin receptor agonist (TPO-RA) approved for the treatment of chronic immune thrombocytopenia (ITP). However, increased thrombogenicity in patients with ITP after initiation of TPO-RA treatment has been reported.Key Clinical Question: We report a case of a patient with ITP who developed a catastrophic antiphospholipid antibody syndrome (CAPS), following treatment with avatrombopag.Clinical Approach: A 20-year-old known chronic patient with ITP presented at the emergency department with a 2-week history of headache, nausea, and abdominal pain, 3 weeks after initiating avatrombopag. In-hospital diagnostic work-up revealed multiple microvascular thrombotic events, including myocardial, cerebrovascular, and pulmonary infarctions. Laboratory test results showed a triple-positive antiphospholipid antibodies serology. Conclusion: The diagnosis of probable avatrombopag-associated CAPS was made.

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