免疫性血栓性血小板减少性紫癜临床误诊分析

Clinical Misdiagnosis of Immune Thrombotic Thrombocytopenia Purpura

目的分析免疫性血栓性血小板减少性紫癜(iTTP)的临床表现、误诊原因、防范误诊措施。方法回顾性分析2001年1月至2023年12月收治的曾误诊为其他疾病的9例iTTP患者的临床资料。结果9例急性起病,表现为典型“三联征”3例,“三联征”合并发热1例,合并肾功能损害1例,典型“五联征”4例。9例误诊为HELLP综合征、弥散性血管内凝血、Evans综合征、溶血尿毒综合征各1例,病毒性脑炎2例,急性肾功能衰竭3例。9例按初诊疾病治疗效果差而转我院,后根据临床症状、外周血红细胞计数、血涂片找红细胞碎片、乳酸脱氢酶、间接胆红素及骨髓象分析、血管性血友病因子裂解蛋白酶13(ADAMTS13)活性及抑制物检测确诊为iTTP。误诊时间3~7 d。9例确诊后均采用治疗性血浆置换,其中7例联合糖皮质激素治疗、2例联合利妥昔单抗治疗。经治疗死亡1例,8例随访1年,病情稳定,均无复发。结论iTTP发病率低,临床表现多样,极易误诊。加强临床医生对该病的认识、提高警惕性,对于高度可疑病例应尽早行ADAMTS13活性及抗体检测,以提高本病的确诊率。

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.