血栓性血小板减少性紫癜临床分析

Clinical Analysis of Thrombotic Thrombocytopenic Purpura

目的 探讨血栓性血小板减少性紫癜（TTP）患者的临床表现、辅助检查特征及治疗方法,以提高TTP的诊断和治疗水平.方法 选择2000年1月至2011年3月,于南昌大学第一附属医院确诊为TTP的12例患者为研究对象.对其临床表现、实验室检查结果、诊治及疗效进行回顾性分析.结果 12例患者中,8例为原发性TTP,4例为继发性TTP.患者典型临床表现：血小板减少、微血管性溶血性贫血、神经精神症状、发热、肾损伤的发生率分别为100％,100％,100％,75％和33％,所有患者均表现为三联征症状,仅4例患者表现为五联征.所有患者的乳酸脱氢酶（LDH）水平均升高,其中LDH水平升高超过正常值上限3倍者为7例,其LDH水平为521～2 936 U/L,平均值为1 258 U/L.5例患者给予血浆置换治疗,其中4例治愈,治愈率为80％（4/5）;7例患者行血浆输注治疗,仅1例治愈,治愈率为14％（1/7）.血浆置换治疗治愈率显著高于血浆输注治疗,且差异有统计学意义（P=0-045）.结论 TTP患者起病多无明显诱因;临床表现以神经精神症状为主;LDH明显升高可作为早期诊断TTP的参考指标;血浆置换是TTP的首选治疗方法.

Objective To investigate the clinical features,auxiliary examination characteristics and treatment means of thrombotic thrombocytopenic purpura （TTP）,in order to improve the level of diagnosis and treatment of TTP.Methods From January 2000 to March 2011,a total of 12 TTP patients in the First Affiliated Hospital of Nanchang University were chosen as the research objects.Their manifestations,laboratory findings,treatment and curative effect were analyzed,retrospectively.Results In these 12 TTP patients,8 cases were idiopathic TTP,4 cases were secondary TTP.The classical clinical manifestations：thrombocytopenia,microangiopathic haemolytic anaemia,neuropsychiatric symptoms,fever,renal dysfunction appeared at the percentage of 100%,100%,100%,75% and 33%,respectively.All patients showed a triad of symptoms,but only 4 patients had the pentad.Lactate dehydrogenase （LDH） levels were increased in all patients,and 7 patients＇ LDH levels reached to the average of 1 258 U/L （521-2 936 U/L）,which increased more than three times of upper limit of normal LDH levels.Five patients were given plasma exchange treatment,including 4 cases were cured,and the cure rate was 80% （4/5）.The rest 7 patients were treated with plasma transfusion,including 1 case were cured,and the cure rate was 14% （1/7）.The cure rate of plasma exchange was higher than that of plasma transfusion.There was statistically significant differences between cure rate of two methods （P =0-045）.Conclusions TTP patients usually have no obvious pathogenesis; the main clinical manifestations is neuropsychiatric symptoms; significantly increase of LDH levels could be used as a reference indicator in the early diagnosis of TTP; and plasma exchange is the preferred treatment for TTP.