Thrombotic Thrombocytopenic Purpura Causing Multiple Large Vessel Occlusions: A Case Report

Thrombotic thrombocytopenic purpura (TTP) is a hematologic disorder characterized by a severe deficiency or absence of the disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13 enzyme (ADAMTS13), which is a protease essential for cleaving von Willebrand factor. This deficiency promotes microvascular thrombosis and can lead to ischemic strokes.

We present the case of a 29-year-old female with a history of rheumatological disease controlled with chronic low-dose prednisone who presented with an acute ischemic stroke due to a large vessel occlusion (LVO). She underwent mechanical thrombectomy, during which extensive recurrent clot formation occurred with recurrent intracranial occlusions. Further evaluation revealed nonbacterial thrombotic endocarditis. Interestingly, her initial hematological workup revealed only fluctuating thrombocytopenia, and her remaining hematologic tests were largely unremarkable. ADAMTS13 level was markedly reduced, and she was ultimately treated with plasma exchange, high-dose corticosteroids, and rituximab, with a good response and no recurrent ischemic events during follow-up.

In this case, we highlight the importance of including hematological disorders like TTP in the differential diagnosis of cryptogenic stroke and particularly in the presence of autoimmune conditions or subtle hematologic abnormalities.