血栓性血小板减少性紫癜患者预后与疗效分析

Analysis of Prognosis and Therapeutic Effect of Patients with Thrombotic Thrombocytopenic Purpura

目的:总结血栓性血小板减少性紫癜(TTP)患者的临床及实验室参数特征,分析疾病预后、急性期疗效的影响因素。方法:回顾性分析湖南省人民医院收治的59例TTP患者的诱因、临床特征、实验室参数、治疗方案等资料,比较各因素在死亡组与生存组的组间差异;分析各因素与预后、急性期疗效的相关性。结果:59例TTP患者中,1名4岁男童为遗传性TTP,其余58例为获得性TTP(39例为特发性TTP,19例为继发性TTP),其中男27例,女31例,中位年龄54(11-84)岁。36例行血管性血友病因子裂解蛋白酶(ADAMTS13)活性测定,34例(94.44%)患者存在酶活性降低,特发性与继发性TTP患者ADAMTS13酶活性程度存在统计学差异(P<0.001)。死亡组、存活组比较,年龄(64岁vs 51岁)、ALT/AST比值(0.61 vs 0.36)、血浆置换(TPE)次数(3次vs 8次)与持续时间(3 d vs 9 d)等因素有统计学差异。多因素分析结果显示,TPE次数(OR=5.175,95%CI:1.169-22.914,P=0.030)、ALT/AST比值(OR=4.387,95%CI:1.019-18.891,P=0.047)与死亡率相关。COX回归分析结果显示,神经精神障碍程度(HR=0.200,95%CI:0.084-0.474,P<0.001)、起病至TPE启动时间(HR=0.288,95%CI:0.114-0.726,P=0.008)、治疗方案(HR=0.336,95%CI:0.125-0.902,P=0.030)与TTP急性期疗效相关。结论:高ALT/AST比值的TTP患者死亡率高;足次、足程血浆置换者死亡率低。重度神经精神障碍、诊治时间延迟、TPE无反应加用利妥昔单抗者疗效欠佳。

Objective:To summarize the features of clinical and laboratory parameters of thrombotic thrombocytopenic purpura(TTP),and to analyze the factors affecting the prognosis and therapeutic effect during the acute phase of the disease.Methods:The etiology,clinical features,laboratory parameters,treatment regimens and other data of 59 TTP patients admitted to Hunan Provincial People's Hospital were retrospectively analyzed.And the differences of each variable between the death group and the survival group were compared,the correlations between each variable and prognosis,as well as the therapeutic effect of the acute phase patients were analyzed.Results:Among the 59 cases of TTP,one 4-year-old boy was inherited TTP,the other 58 cases were acquired TTP(39 cases were idiopathic TTP and 19 were secondary TTP),including 27 males and 31 females,with a median age of 54(11-84)years old.36 patients were tested for von Willebrand factor cleaving protease(ADAMTS13)activity,and 34 patients(94.44%)had decreased ADAMTS13 activity.There was a statistical difference in the activity of ADAMTS13 between idiopathic and secondary TTP patients(P<0.001).There were statistical differences in factors such as age(64 vs 51 years),ALT/AST ratio(0.61 vs 0.36),therapeutic plasma exchange(TPE)times(3 times vs 8 times),and TPE duration(3 d vs 9 d)between the death and survival groups.Multivariate analysis showed that total times of TPE(OR=5.175,95%CI:1.169-22.914,P=0.030),ALT/AST ratio(OR=4.387,95%CI:1.019-18.891,P=0.047)were associated with mortality.COX regression analysis showed that degree of neuropsychiatric disorder(HR=0.200,95%CI:0.084-0.474,P<0.001),days from onset to initiation of TPE(HR=0.288,95%CI:0.114-0.726,P=0.008),treatment regimen(HR=0.336,95%CI:0.125-0.902,P=0.030)were associated with platelet recovery and therapeutic effect in the acute phase of the patients.Conclusion:TTP patients with high ALT/AST ratio have a higher mortality rate.Patients with enough times and full course of TPE have lower mortality rate.Poor therapeutic effect were found in TTP patients with severe neuropsychiatric disorders,delayed diagnosis and treatment,and who added rituximab when TPE was ineffective.