免疫性血栓性血小板减少性紫癜临床误诊分析OACSTPCD

Objective To analyze the clinical manifestations,causes of misdiagnosis and preventive measures of im-mune thrombotic thrombocytopenic purpura(iTTP).Methods The clinical data of 9 patients with iTTP who had been misdi-agnosed with other diseases from January 2001 to December 2023 were retrospectively analyzed.Results In 9 patients with acute onset,there were 3 patients with typical＂triad＂,1 patient with＂triad＂ combined with fever,1 patient with renal func-tion impairment,4 patients with typical ＂pentad＂.Of 9 patients,there was misdiagnosis as HELLP syndrome(n=1),dis-seminated intravascular coagulation(DIC,n=1),Evans syndrome(n=1),hemolytic uremic syndrome(HUS,n=1),vi-ral encephalitis(n=2),acute renal failure(n=3).Nine patients were transferred to our hospital due to the poor therapeutic effect according to the newly diagnosed disease,and iTTP was confirmed according to clinical symptoms,peripheral blood red blood cell(RBC)count,RBC fragments found by smear,lactate dehydrogenase,indirect bilirubin,bone marrow image anal-ysis,a disintegrin like and metalloproteinase with thrombospondin type 1 motif 13(ADAMTS13)activity and inhibitor detec-tion.The misdiagnosis lasted 3-7 d.Therapeutic plasmapheresis was used in all 9 patients after diagnosis,including 7 patients receiving combined glucocorticoid and 2 patients receiving combined Rituximab.After treatment,1 patient died,and the other 8 patients were followed up for 1 year,with stable condition and without recurrence.Conclusion The incidence of iTTP is low,and the clinical manifestations are diverse,which often lead to misdiagnosis.It is necessary to strengthen clinicians'awareness and vigilance of the disease,and ADAMTS13 activity and antibody detection should be performed as soon as possi-ble for highly suspicious cases,to improve the diagnosis rate of the disease.