What question did this study set out to answer?

To highlight the rare presentation of thrombotic thrombocytopenic purpura (TTP) in a patient with neurological symptoms and absence of schistocytes.

May 20, 2026

A102-08 Cerebral Confusion: A Rare Presentation of Thrombotic Thrombocytopenic Purpura in a Patient With Stroke-Like Features

Key Points

To highlight the rare presentation of thrombotic thrombocytopenic purpura (TTP) in a patient with neurological symptoms and absence of schistocytes.
48-year-old male admitted with stroke-like symptoms and severe thrombocytopenia
Extensive workup including MRI and ADAMTS13 testing
Initiated treatment with plasmapheresis and immunosuppressive therapy upon TTP diagnosis.
Confirmed diagnosis of TTP with low ADAMTS13 activity and positive inhibitor
Patient's mentation improved within four days post-therapy
Full recovery achieved, enabling return to normal activities.

Abstract

Abstract Introduction Early recognition and treatment of thrombotic thrombocytopenic purpura (TTP) is essential, as untreated cases carry a mortality rate exceeding 90%, particularly in patients with severe neurological involvement, cardiac events, or delayed therapeutic response. Although acquired TTP is rare, affecting only about 6 individuals per million annually, its presentation can resemble other causes of thrombocytopenia such as disseminated intravascular coagulation, immune thrombocytopenic purpura, and hemolytic uremic syndrome, potentially delaying diagnosis and increasing the risk of widespread microvascular thrombosis. Classically, TTP is associated with the “pentad” of microangiopathic hemolytic anemia, severe thrombocytopenia, fever, neurological abnormalities, and renal dysfunction; however, this complete constellation of findings is seldom observed in practice. Even more unusual is the absence of schistocytes in TTP patients. Description We present the case of a 69-year-old male admitted with left facial and upper extremity numbness, with MRI showing widespread punctate infarcts. His hospital course was complicated by new-onset seizures, severe thrombocytopenia requiring transfusion, and worsening mentation necessitating intubation and ICU transfer. An extensive workup for thrombocytopenia was unrevealing, and hematology initiated IVIG for presumed ITP without improvement. Despite a low PLASMIC score due to absent schistocytes and underlying end stage renal disease, ADAMTS13 testing was pursued due to high clinical suspicion of TTP. This revealed very low activity with a positive inhibitor confirming TTP and prompting initiation of plasmapheresis, Rituximab, Caplacizumab (Cablivi), and steroids. The patient’s mentation improved rapidly, enabling extubation four days after starting therapy. He ultimately made a full recovery and was discharged home, returning to his normal daily activities. Discussion Although TTP is rare, prompt recognition and treatment are crucial to patient survival. This case underscores a rare clinical picture of TTP without schistocytes, making the initial diagnosis challenging. Furthermore, the application of risk stratification tools such as the PLASMIC score was not suggestive of TTP in this patient. Importantly, therapy for TTP should never be delayed while awaiting confirmatory testing, as ongoing microvascular thrombosis can rapidly cause irreversible organ damage, particularly to the brain, heart, and kidneys. Once clinical suspicion is high enough, rapid initiation of plasma exchange, immunosuppression, and adjunctive therapies are central to improving outcomes. Equally important is vigilant monitoring for complications and relapse, as recurrence occurs in up to 40% of patients with acquired TTP. Ultimately, early diagnosis, timely treatment, and long-term follow-up are essential not only for survival but also for reducing morbidity and preventing disease recurrence. This abstract is funded by: None

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