血栓性血小板减少性紫癜2例报道

摘要

目的 总结兰州大学第二医院2例血栓性血小板减少性紫癜(TTP)的所有实验检测资料.方法 回顾性分析与TTP诊断特征密切相关的实验室检测:血红蛋白(Hb)、血小板(PLT)、网织红细胞(RET)、裂片红细胞(FRC)、尿蛋白(PRO)、尿潜血(BLD)、乳酸脱氢酶(LDH)、总胆红素(TBil)、直接胆红素(DBil)、间接胆红素(IBil)、尿素氮(BUN),肌酐(Cr)、D二聚体(D-D)及骨髓检测,并归纳分析.结果 血浆、PLT、静丙输注无法改善PLT的严重减少；输注红细胞(RBC),Hb进行性下降；尿液PRO及BLD定性持续阳性；溶血检测中的RET、TBil、IBil及LDH持续增高；凝血检测中D-D持续不降；骨髓分析提示2例患者分别是增生性贫血与溶血性贫血并考虑Evans综合症.结论 对于TTP浅显认识导致临床诊治延误.回顾性总结TTP实验室检测及临床特征,发现有经典“五联征”,包括血小板减少症、微血管病性溶血性贫血、神经症状、肾脏损害及发热.%Objective To summarize the examination data of 2 cases with thrombotic thrombocytopenic purpura (TTP)in Lanzhou University Second Hospital. Methods The closely related diagnostic features of TTP were reviewed retrospectively:hemoglobin (Hb), platelet (PLT), reticulocyte (RET), fragments of red blood cell (FRC), urinary protein (PRO ), urinary occult blood (BLD ), lactate dehydrogenase (LDH), total bilirubin (Tbil), direct bilirubin (Dbil),indirect bilirubin (Ibil),urea nitrogen (BUN),creatinine (Cr),D-dimer (D-D)and bone marrow analysis. Results Intravenous infusing plasma, PLT and immunoglobulin could not improve the severe reducing of PLT. Intravenously infusing red blood cells (RBC), Hb declined progressively. PRO and BLD were persistently positive. RET,Tbil,Ibil and LDH of hemolysis test continued to rise. D-D of coagulation test continued not to reduce. Bone marrow analysis showed that 2 patients were proliferative anemia and hemolytic anemia with Evans syndrome respectively. Conclusions Shallow understanding of TTP diagnosis leads to delay the clinical diagnosis and treatment. By retrospectively reviewing the tests and clinical features of TTP, the classic " five signs" are found, including thrombocytopenia,microangiopathic hemolytic anemia,neurologic symptoms,renal damage and fever.