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出血倾向、肝脾肿大、肠出血、腹痛、抽搐、猝死、乏力、蛋白尿、鼻出血
Thrombotic thrombocytopenic purpura (TTP) is thrombotic microangiopathy due to ADAMTS13 deficiency (autoimmune/inherited), causing microvascular thrombi. Pentad: fever, thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms, renal dysfunction. Treatment: emergent plasma exchange (PLEX) daily until remission; glucocorticoids; rituximab for refractory/relapsing; caplacizumab for acquired TTP; immunosuppressants; supportive (transfusions cautiously, avoid platelet transfusion unless life-threatening bleeding); monitor ADAMTS13, LDH, platelets, schistocytes; follow-up hematology.
Purpura; fatigue; fever; neurologic symptoms; renal dysfunction
ADAMTS13 deficiency causes ultra-large vWF multimers and microvascular thrombi.
Emergent PLEX; glucocorticoids; rituximab; caplacizumab; supportive
Early diagnosis; monitor ADAMTS13; avoid triggers.
It is recommended to visit the Hematology/Critical-care department; an appointment can be booked on this platform before the visit.
Warm reminder: The above information is for health science reference only and cannot replace professional medical diagnosis. If you have related symptoms, please seek medical attention at a regular hospital promptly.
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