血浆疗法联合小剂量利妥昔单抗治疗初发获得性TTP

A clinical study on incipient patients with acquired thrombotic thrombocytopenic purpura treated with plasma therapy and low-dose rituximab

目的探讨血浆疗法联合小剂量利妥昔单抗治疗初发获得性血栓性血小板减少性紫癜（TTP）的疗效。方法回顾性分析6例初发获得性TTP患者的临床特征、实验室检查,观察血浆疗法联合小剂量利妥昔单抗治疗反应及转归。结果本组6例获得性TTP患者中,4例有诱发因素,包括自身免疫性异常、系统性红斑狼疮及抗磷脂抗体综合征。初诊时仅1例表现为典型＂五联＂征,5例表现为＂三联＂征;5例网织红细胞百分比升高,6例均检出破碎红细胞,其中,1例同时检出小球形红细胞;6例初诊时乳酸脱氢酶均升高,4例总胆红素升高,但仅1例肌酐升高。6例患者均接受了血浆疗法（血浆置换或/和血浆输注）联合小剂量利妥昔单抗（100 mg每周1次,连用4次）治疗,结果 6例患者均达CR,中位随访12（6~18）个月,6例患者均无复发。结论 TTP早期诊断依赖临床资料的综合分析,血浆疗法联合小剂量利妥昔单抗可有效治疗初发获得性TTP。

Objective To enhance the understanding of thromboticthrombocytopenic purpura （TTP） and to evaluate the efficacy of plasma therapy and low-dose rituximab in treating incipient patients with acquired TTP, and the clinical data of acquired TTP were investigated. Methods The clinical features,laboratory characteristics, treatment and outcome of six patients with TTP were retro- spectively analyzed and investigated. All 6 cases received plasma therapy and low-dose rituximab which was administered at the dose of 100 mg once weekly, for 4 consecutive weeks. Results Of all 6 patients with TTP,4 cases had causative factors, including abnor- mal autoimmune, systemic 1 upus erythematosus （SLE）and antiphospholipid antibody syndrome （APS）. When the patients were diag- nosed primarily, only one of them had typical pentalogy of TTP, while, the others had triad. Five patients had high percentage of retie- ulocyte. The schistocytes of were detected in the peripheral blood smears of all six patients. One patient with schistocytes was found microspherocyte in his peripheral blood smears. Six patients had high level of lactate dehydrogenase. Four patients had high level of total biliruhin. Only one patient had high level of ereatinine. As a result, all six patients achieved complete remission （CR）. During the follow-up （ median ： 12 months ; range ： 6-18 months ） , no patient experienced relapse. Conclusion The early diagnosis of TTP was based on comprehensive analysis of clinical features. Treatment with plasma therapy and low-dose rituximab is effective for in- cipient patients with acquired TTP.