Thrombotic Thrombocytopenic Purpura as a Rare Stroke Mimic

Figure

Blood smear of the patient with masses (89 per thousand, normal range: up to 5 per thousand) of fragmentocytes (arrows).

A 37-year-old female patient presented to our emergency department with an about 30-minute history of right-sided hemiparesis, mainly affecting her arm, and severe aphasia with the suspected diagnosis of stroke. Computed tomography findings ruled out an intracranial bleeding and occlusion of a large vessel. In the presence of initially unexplained severe anemia (hemoglobin: 6.5 g/dL), lysis therapy was not performed. Laboratory testing found increased hemolysis parameters as well as marked thrombocytopenia (10/nL), with masses of fragmentocytes in the blood smear (Figure). The patient was transferred to a university medical center as an emergency case with a suspected diagnosis of thrombotic thrombocytopenic purpura (TTP), where she immediately underwent plasmapheresis and was treated with steroids and caplacizumab. With this regimen, the neurological symptoms subsided completely. The diagnosis of TTP was confirmed by the detection of autoimmune-mediated ADAMTS13 deficiency. The resulting microthrombi lead to hemolysis (“fragmentocytes“) and impaired microcirculation, manifesting, among other symptoms, as transient neurological deficits as in our case, but can also manifest as cerebral ischemia. TTP is a rare but time-critical emergency with a fatal outcome in 90% of cases, if left untreated.

Acknowledgement: We would like to thank the III Medical Clinic of University Medicine Mainz for the excellent collaboration and promptly taking over the patient’s care.

Dr. med. Juri Mattern, Interdisziplinäre Zentrale Aufnahme, Diakonissen-Stiftungs-Krankenhaus Speyer, [e-mail]

Dr. med. Björn Jacobi, Prof. Dr. med. Markus Munder, Klinik für Innere Medizin- Schwerpunkt Hämatologie und internistische Onkologie, Diakonissen-Stiftungs-Krankenhaus Speyer

Conflict of interest: The authors declare no conflict of interest.

Translated from the original German by Ralf Thoene, MD.

Cite this as: Mattern J, Jacobi B, Munder M: Thrombotic thrombocytopenic purpura as a rare stroke mimic. Dtsch Arztebl Int 2025; 122: 369. DOI: 10.3238/arztebl.m2025.0046