2026-07-10 | Secondary thrombotic thrombocytopenic purpura with features of Evans syndrome in a patient with SLE.

A female in her early 40s with systemic lupus erythematosus (SLE) and prior immune thrombocytopenic purpura presented with dizziness, dyspnoea, haematuria and fatigue. Investigations revealed a life-threatening thrombotic microangiopathy triad (platelets 3000/µL, microangiopathic haemolytic anaemia, acute kidney injury) and the strongly positive Coombs test with haemolysis markers supported autoimmune haemolysis in addition to microangiopathic haemolysis. Together with her prior history of immune thrombocytopenia, these findings are consistent with an Evans syndrome overlap, though consumptive thrombocytopenia from thrombotic thrombocytopenic purpura (TTP) could not be excluded. There was evidence of SLE vasculitis. Despite diagnostic overlap obscuring TTP, empiric plasma exchange (PLEX), steroids and rituximab induced rapid haematologic and clinical recovery. Days later, severe ADAMTS13 deficiency (<0.03 IU/mL) confirmed SLE-associated secondary TTP. This case highlights the critical need for empiric PLEX in suspected TTP when possible Evans syndrome complicates SLE and the efficacy of combined immunomodulation targeting both entities.

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2026-07-09 | Update in treatment options for congenital and immune thrombotic thrombocytopenic purpura.

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy resulting either from congenital deficiency (cTTP) or acquired (immune) deficiency (iTTP) of A Disintegrin and Metalloprotease with ThromboSpondin-type 1 motif, member 13 (ADAMTS13). Deficiency of ADAMTS13 leads to disseminated platelet thrombosis and organ dysfunction. High mortality of cTTP is prevented by plasma infusion to replace the deficient protease, or more recently by infusion of recombinant ADAMTS13. Standard treatment of iTTP includes steroids, plasma exchange, and rituximab, with or without caplacizumab. Although standard treatment of iTTP improves mortality, refractory cases persist, indicating the need for additional treatment options. This review summarizes the status of novel treatment options for cTTP and iTTP, including additional recombinant ADAMTS13 products, ADAMTS13 gene therapies, plasma cell-directed therapies (bortezomib, daratumumab) as well as novel inhibitors of von Willebrand factor activity.

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2026-07-09 | Influenza A-Associated Thrombotic Microangiopathy With Normal a Disintegrin and Metalloproteinase With Thrombospondin Type 1 Motif 13 (ADAMTS13) in a Young Male Patient: A Case Report.

Thrombotic microangiopathy (TMA) is a life-threatening syndrome characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end-organ injury resulting from widespread microvascular thrombosis. It encompasses several entities, including thrombotic thrombocytopenic purpura (TTP), atypical hemolytic uremic syndrome (aHUS), and secondary forms triggered by infections, medications, or systemic disease. Viral infections, including influenza A, have been increasingly recognized as rare triggers of secondary TMA, through endothelial injury and immune dysregulation, even in the absence of severe a disintegrin and metalloproteinase with thrombospondin type 1 motif 13 (ADAMTS13) deficiency. We report a case of a 21-year-old male patient who presented with fever, productive cough, and cola-colored urine following a positive influenza A antigen test. He was found to have severe thrombocytopenia, microangiopathic hemolytic anemia with schistocytes on peripheral smear, markedly elevated lactate dehydrogenase, acute kidney injury, and concurrent rhabdomyolysis. ADAMTS13 activity was 0.8 IU/mL (equivalent to 80% activity), within the normal range, and consistent with secondary influenza A-triggered TMA. The patient was treated with therapeutic plasma exchange and high-dose intravenous methylprednisolone, achieving full hematological recovery. This case highlights the importance of recognizing influenza A as a trigger of secondary TMA with a TTP-like phenotype despite normal ADAMTS13 activity, and underscores the critical role of early empiric plasma exchange as a life-saving intervention.

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