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目的:探讨血栓性血小板减少性紫癜(TTP)的临床特点和治疗策略,提高TTP的诊疗水平。方法:回顾性分析57例TTP患者的临床表现、实验室检查、治疗方法、疗效和预后。结果:57例患者中,男16例,女41例,中位发病年龄38岁,49例表现为三联征,22例表现为五联征。血小板、血红蛋白及乳酸脱氢酶均值分别为(16.83±13.79)×109/L,(65.11±19.01)g/L和(1 169.69±668.27)U/L。外周血红细胞碎片比例为1.3%~31.0%。使用血浆置换+糖皮质激素及血浆置换+糖皮质激素+免疫抑制剂的有效率分别为71.43%(25/35)和100%(13/13);9例难治或复发的患者加用利妥昔单抗治疗,有效率为100%(9/9)。57例患者中,9例死亡,11例复发。死亡组与存活组在血红蛋白(P=0.027)、乳酸脱氢酶(P=0.024)、尿素氮(P=0.004)、年龄(P=0.009)、严重神经系统症状(P=0.037)、昏迷(P=0.023)和神经系统症状不断加重(P=0.000)方面差异有统计学意义,其中神经系统症状不断加重是TTP的独立预后因素(P=0.025)。复发组与非复发组年龄差异有统计学意义(P=0.005)。结论:TTP患者多数以三联征起病,血浆置换联合糖皮质激素是其标准治疗方案。对于难治复发的患者,利妥昔单抗可以提高有效率。神经系统症状不断加重是TTP的不良预后因素。
Objective: To investigate the clinical features and treatment strategies of thrombotic thrombocytopenic purpura (TTP) and to improve the diagnosis and treatment of TTP. Methods: A retrospective analysis of 57 cases of TTP clinical manifestations, laboratory tests, treatment, efficacy and prognosis. Results: Of the 57 patients, 16 were male and 41 were female, with a median age of 38 years. Forty-nine patients showed triple sign and 22 had levy. The average values of platelet, hemoglobin and lactate dehydrogenase were (16.83 ± 13.79) × 109 / L, (65.11 ± 19.01) g / L and (1 169.69 ± 668.27) U / L, respectively. Peripheral blood red cell debris ratio of 1.3% ~ 31.0%. The effective rates of plasma exchange + glucocorticoids and plasma exchange + glucocorticoids + immunosuppressive agents were 71.43% (25/35) and 100% (13/13), respectively. Nine patients with refractory or relapsed Toximab treatment, the effective rate was 100% (9/9). Of 57 patients, 9 were fatal and 11 were relapsed. The mortality and survival groups were significantly higher in hemoglobin (P = 0.027), lactate dehydrogenase (P = 0.024), urea nitrogen (P = 0.004), age (P = 0.009), severe neurological symptoms (P = 0.037) P = 0.023) and worsening neurological symptoms (P = 0.000). The worsening neurological symptoms were the independent prognostic factors of TTP (P = 0.025). There was a significant difference in age between the recurrent and non-recurrent groups (P = 0.005). Conclusion: The majority of TTP patients with triad symptoms, plasma exchange with glucocorticoid is its standard treatment. Rituximab improves efficiency in patients refractory to relapse. The worsening neurological symptoms are a poor prognostic factor for TTP.