血栓性血小板减少性紫癜的临床诊治

目的通过分析血栓性血小板减少性紫瘢(TTP)的临床特征及治疗经过,加强对TTP的认识。方法对10例TTP患者的临床表现、实验室检查及治疗情况进行回顾性分析。结果经过血浆置换、肾上腺糖皮质激素和环孢素等治疗后,9例患者完全缓解,1例死亡(病例6死亡)。9例好转患者经血浆置换治疗3次后均神志转清(病例2、病例9血浆置换一次后精神症状消失)、血小板计数及血清LDH下降。9例好转的患者中有1例联合环孢素维持治疗。在获得完全缓解的9例患者的随访过程中,病例4于初次治疗后2年复发、病例2半年内复发2次。结论 TTP是由于血小板性微血栓形成而造成全身多系统、多脏器功能障碍的一种血栓性微血管病,临床过程凶险,且死亡率极高,早期诊断和血浆置换联合糖皮质激素为主的治疗可挽救患者生命。

Clinical diagnosis and treatment of thrombotic thrombocytopenic purpura

Objective To strengthen the understanding of thrombotic thrombocytopenic purpura(TTP) by analyzing its clinical characteristics and treatment process. Methods The clinical manifestations, laboratory examination and treatment of 10 cases of TTP were retrospectively analyzed. Results After plasma exchange, glucocorticoid and cyclosporine treatment, 9 patients were completely remitted and 1 patient died(case 6 death). All the 9 improved patients were cleared of consciousness after 3 times of plasma exchange(mental symptoms disappeared after 1 plasma exchange in case 2 and case 9), and their platelet counts and serum LDH decreased. One of the 9 patients who improved was treated with cyclosporine maintenance therapy.During the follow-up of the 9 patients with complete remission, case 4 relapsed 2 years after the initial treatment, and case 2 relapsed twice in half a year. Conclusion TTP is a thrombotic microangiopathy with multiple systemic and multiple organ dysfunction caused by platelet microthrombosis. The clinical process is dangerous and the mortality rate is very high. Early diagnosis and plasma exchange combined with glucocorticoid therapy can save patients’ lives.