Refractory Thrombotic Thrombocytopenic Purpura

Background: Thrombotic thrombocytopenic purpura (TTP) is a rare, lifethreatening thrombotic microangiopathy characterized by thrombocytopenia, microangiopathic hemolytic anemia, and severe ADAMTS13 deficiency, often due to inhibitory autoantibodies.If left untreated, the mortality rate can exceed 90%.Case Report: A 44-year-old male presented with fever, confusion, agitation, and pallor.Laboratory evaluation revealed DAT-negative hemolytic anemia, thrombocytopenia, schistocytes on peripheral smear, elevated LDH and creatinine, and neurologic symptoms.His PLASMIC score was high, suggesting severe ADAMTS13 deficiency, later confirmed by a markedly reduced ADAMTS13 activity level (<0.03IU/mL) and the presence of anti-ADAMTS13 antibodies.The patient was promptly treated with daily therapeutic plasma exchange (TPE) and high-dose methylprednisolone.Due to delayed platelet recovery, rituximab was added, resulting in sustained hematologic improvement.Conclusion: This case highlights the importance of early recognition and prompt initiation of plasma exchange and immunosuppressive therapy in suspected TTP.The addition of rituximab in refractory cases may hasten recovery and reduce the risk of relapse.Timely diagnosis and aggressive treatment are critical to improving outcomes in patients with acquired TTP.