内科学课件：34_TTP

1、Hemorrhagic Disorder Ye Jieyu Department of Hematology Nanfang Hospital,My God ! T39.5， Twitching! Gnathospasmus! Hyperemesis!,Nervous system disease: infection?,lumbar puncture CT scan,CT scan: Normal,Nervous system infection?,cerebrospinal fluid(CSF): Normal,What ?! PLT 10G/L，HGB 86g/L Purpura! An

2、emia!,Evans Syndrome? Hemolytic exam Auto-antibodies exam ITP? Hemolytic exam Bone marrow aspiration DIC? Coagulation exam,What will be suspect?,Auto-antibodies exams Negative,Coagulation exam Normal,Results,Free hemoglobin 185mg/L,Evans Syndrome? ITP? DIC? ,Intravascular hemolysis,Let me guess! Mic

3、roangiopathic hemolytic anemia (MIHA)?,What will be the next?,Really! Thrombic Thrombocytopenic Purpura (TTP)!,Thrombic Thrombocytopenic Purpura,What is TTP? How to diagnosis TTP? How to treat TTP?,What is TTP?,Disseminated thrombotic microangiopathy Symptoms including thrombocytopenia purpura, micr

4、oangiopathic hemolytic anemia, fluctuating neurological signs, renal impairment, and fever -”Classical pentad” Rapid progress, poor prognosis ADAMTS13 deficiency is the major pathogenesis,/action/showMediaPlayer?doi=10.1056%2FNEJMra1312353&aid=NEJMra1312353_attach_1&area= viedio.MP

5、4,ADAMTS13 deficiency and TTP,Mechanism of thrombocytopenia and MIHA,Thrombocytopenia: Sequestration of platelets in microvascular thrombi,MIHA: fragmentation of erythrocytes during passage through the meshwork,How to diagnosis TTP,A combination of clinical history, examination of the patient, and a

6、 blood smear ADAMTS13 activity assays assist in confirming the diagnosis,DIC? But without coagulation abnormality,ITP? But without neurological signs,Evans? But Coombs(-), schistocytes2%,Neurological signs? But with thrombocytopenia and anemia,SLE? But autoantibodies (-),Diagnosis of TTP,How to treat TTP?,Life-threatening disease, easy