Background:thrombotic thrombocytopenic purpura(TTP)is an autoimmune disease with high mortality.An AD AMTS 13(a disintegrin and metallopro-tection with a thrombospondin type 1 motif,member 13)activity level of less th...Background:thrombotic thrombocytopenic purpura(TTP)is an autoimmune disease with high mortality.An AD AMTS 13(a disintegrin and metallopro-tection with a thrombospondin type 1 motif,member 13)activity level of less than 10% supports the diagnosis of TTP in appropriate clinical contexts.Historically,nearly all patients died during the first month of illness with severe hemolytic anemia,abundant schistocytes,profound thrombocytopenia,neurological deficits,renal injury,and fever.Most such patients have severe ADAMTS13 deficiency.Currently,the first-line treatment of TTP including plasma exchange therapy and preemptive B-cell depletion with rituximab can increase the survival rate and reduce the recurrence rate.Case presentation:a 34-year-old male patient who presented with hemato-chezia and progression of disorder of consciousness was misdiagnosed with esophageal and gastric varices bleeding and hepatic encephalopathy based on his history of hepatitis B.However,he was diagnosed with TTP based on clinical and laboratory results.He received comprehensive and systemic treatments including taking methylprednisolone,rituximab,and multiple sessions of plasmapheresis.The patient recovered well after active treatment.Conclusion:in this article,we reported a patient diagnosed as TTP and reviewed the disease characteristics,pathogenesis,and treatment of TTP in order to deepen the understadning of TTP and to reduce its missed diagnosis and misdiagnosis.展开更多
文摘Background:thrombotic thrombocytopenic purpura(TTP)is an autoimmune disease with high mortality.An AD AMTS 13(a disintegrin and metallopro-tection with a thrombospondin type 1 motif,member 13)activity level of less than 10% supports the diagnosis of TTP in appropriate clinical contexts.Historically,nearly all patients died during the first month of illness with severe hemolytic anemia,abundant schistocytes,profound thrombocytopenia,neurological deficits,renal injury,and fever.Most such patients have severe ADAMTS13 deficiency.Currently,the first-line treatment of TTP including plasma exchange therapy and preemptive B-cell depletion with rituximab can increase the survival rate and reduce the recurrence rate.Case presentation:a 34-year-old male patient who presented with hemato-chezia and progression of disorder of consciousness was misdiagnosed with esophageal and gastric varices bleeding and hepatic encephalopathy based on his history of hepatitis B.However,he was diagnosed with TTP based on clinical and laboratory results.He received comprehensive and systemic treatments including taking methylprednisolone,rituximab,and multiple sessions of plasmapheresis.The patient recovered well after active treatment.Conclusion:in this article,we reported a patient diagnosed as TTP and reviewed the disease characteristics,pathogenesis,and treatment of TTP in order to deepen the understadning of TTP and to reduce its missed diagnosis and misdiagnosis.
