Pancreatic Cancer Presenting with Thrombotic Thrombocytopenic Purpura: A Case Report and Review of Literature

Abstract Thrombotic thrombocytopenic purpura (TTP) is a rare form of thrombotic microangiopathy (TMA). Cancer-related TMA typically arises from microvascular metastasis and bone marrow involvement. ADAMTS13 is a plasma protease that regulates von Willebrand factor and prevents platelet aggregation and microvascular thrombosis, with clinical value in diagnosing TTP. While cancer-related TMA typically presents with normal ADAMTS13 levels, some patients have severely reduced ADAMTS13 levels, consistent with cancer-related TTP. Potential mechanisms include immunomediated ADAMTS13 deficiency, endothelial damage from bone marrow metastasis, and paraneoplastic processes. Differentiating cancer-related TTP from other types of TMA is challenging due to overlapping clinical features; however, severely decreased ADAMTS13 activity (<10%) and high PLASMIC score, a predictor of severe ADAMTS13 deficiency, can help to identify TTP. Timely initiation of plasma exchange is critical for TTP management, while cancer-related TMA generally does not benefit from plasma exchange. Here, we report a case of metastatic pancreatic cancer presenting with TTP in whom the rapid initiation of plasma exchange and systemic chemotherapy led to the resolution of TTP. The patient continues to receive systemic chemotherapy with regular follow-up, highlighting the importance of early diagnosis and tailored treatment in improving outcomes.