Immune-Mediated Thrombotic Thrombocytopenic Purpura in a Patient With Systemic Lupus Erythematosus and Recent Epstein-Barr Virus Infection: A Diagnostic Challenge.

Document Type

Article

Publication Date

6-1-2026

Abstract

Patients with systemic lupus erythematosus (SLE) can develop thrombocytopenia from multiple etiologies. It is important not to miss immune-mediated thrombotic thrombocytopenic purpura (TTP) as a cause of severe thrombocytopenia because untreated TTP can rapidly progress to multiorgan failure and death. The diagnosis is often challenging because autoimmune cytopenias and concurrent infections may mimic TTP. We present a case of ADAMTS13-confirmed immune-mediated TTP in a patient with SLE and recent Epstein-Barr virus (EBV) infection initially suspected to have immune thrombocytopenia. Progressive microangiopathic hemolytic anemia with worsening schistocytosis and a high PLASMIC score prompted urgent initiation of plasma exchange before ADAMTS13 confirmation. The patient was successfully treated with plasma exchange, corticosteroids, rituximab, and caplacizumab.

Publication Title

Cureus

Volume

18

Issue

6

First Page

110029

Last Page

110029

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