SHM Converge Scientific Abstract ePoster Gallery-Autoimmune TTP Identified After Recurrent Presentations With Cryptogenic Ischemic Stroke

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This case describes a 54-year-old woman with recurrent cryptogenic ischemic strokes who presented to the ED with acute right leg weakness. Her history included fibromuscular dysplasia of the bilateral internal carotid arteries, chronic thrombocytopenia previously thought to be immune thrombocytopenic purpura, and inherited thrombophilia mutations (Factor V Leiden and prothrombin G20210A).<br /><br />On admission, MRI showed multifocal acute infarcts, while labs demonstrated worsening thrombocytopenia, new normocytic anemia, acute kidney injury, elevated LDH, undetectable haptoglobin, and rare schistocytes on peripheral smear, suggesting thrombotic microangiopathy. Because the infarcts appeared embolic, a transesophageal echocardiogram was performed early and showed an 8 x 13 mm mitral valve mass. This initially seemed to explain the strokes, raising concern for non-bacterial thrombotic endocarditis or embolization from the valve lesion.<br /><br />However, progressive thrombocytopenia and persistent renal dysfunction prompted further evaluation for thrombotic microangiopathy. The patient’s PLASMIC score was 5, indicating intermediate risk for severe ADAMTS13 deficiency. Subsequent testing showed ADAMTS13 activity of 5% with an ADAMTS13 inhibitor, confirming autoimmune thrombotic thrombocytopenic purpura (TTP).<br /><br />She was treated with six sessions of therapeutic plasma exchange followed by rituximab, resulting in complete resolution of thrombocytopenia and improvement in ADAMTS13 activity to 65% two months after discharge.<br /><br />The report emphasizes that TTP can present without the full classic pentad and may be missed when another plausible stroke mechanism is identified. Clinicians should consider TTP in patients with acute neurologic deficits, thrombocytopenia, and hemolysis, and use the PLASMIC score to guide urgent plasma exchange, even when an alternative diagnosis seems convincing.

Asset Subtitle

Allison A. Reinhardt

Meta Tag

Author List Allison A. Reinhardt, Aneel A. Ashrani, Bibek Saha, Dhairya M. Jarsania, Katlin Schmitz, Nickolas Steinauer

Category Clinical Vignettes

Concept Immune-mediated Thrombotic Thrombocytopenic Purpura

Concept ADAMTS13 Deficiency

Concept Thrombocytopenia

Concept Hemolysis

Concept PLASMIC Score

Distinguished Non-Finalist

Presenter Organization Mayo Clinic

Presenting Author Allison A. Reinhardt

Track Adult

Keywords

thrombotic thrombocytopenic purpura

ischemic stroke

thrombotic microangiopathy

ADAMTS13 deficiency

plasma exchange

rituximab

thrombocytopenia

hemolysis

mitral valve mass

PLASMIC score

Immune-mediated Thrombotic Thrombocytopenic Purpura

ADAMTS13 Deficiency

Thrombocytopenia

Hemolysis

PLASMIC Score