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East Florida


HCA Florida Kendall Hospital


Internal Medicine

Document Type


Publication Date



thrombotic thrombocytopenic purpura, TTP, drug-related side effects and adverse reactions


Critical Care | Internal Medicine | Medicine and Health Sciences


A 54-year-old man with a medical history notable for hypertension and type 2 diabetes mellitus presented with altered mental status. Initial assessment revealed altered consciousness, aphasia, and diffuse generalized petechiae. Having recently migrated from Colombia, he had received four doses of the Pfizer COVID-19 vaccine, with the last dose administered one month prior to presentation. Laboratory results indicated severe thrombocytopenia (platelet count: 3), microangiopathic hemolytic anemia, acute myocardial, liver, and kidney injury, prompting a presumptive diagnosis of thrombotic thrombocytopenic purpura (TTP). Confirmation was achieved through an ADAMTS13 level of less than 2 percent. Despite initiating high-dose solumedrol and emergent plasmapheresis, the patient's neurological status deteriorated six days into treatment, necessitating mechanical ventilation, suggestive of treatment resistance, confirmed by persistent ADAMTS13 levels under 2 percent. In addition to high-dose steroids and daily plasmapheresis, Rituximab was introduced due to poor prognosis and refractoriness. Remarkably, Caplacizumab, a Von Willebrand factor-directed monoclonal antibody, was initiated alongside the aforementioned treatments, resulting in notable clinical improvement. The patient's neurological status fully recovered, along with resolution of end organ damage, underscoring the potential of Caplacizumab in treating refractory thrombotic thrombocytopenic purpura induced by COVID-19 vaccination or the virus itself.

This case underscores the emergence of acquired refractory TTP due to COVID-19 vaccination or viral exposure, emphasizing the significance of early identification through comprehensive vaccination history and titers. Furthermore, the successful application of Caplacizumab highlights its efficacy in managing refractory thrombotic thrombocytopenic purpura.

Where’s ADAM? Successful Treatment of COVID-19 Vaccine-Related Refractory Thrombotic Thrombocytopenic Purpura



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