Thrombotic thrombocytopenic purpura (TTP) is a classic, but not a common disorder of hematology. Plasma exchange (PE) was shown to nearly reverse its 90% mortality rate. However, there are still some fatal outcomes in this dramatic disease. We present our experience of plasma exchange plus corticosteroids for the treatment of TTP in our hospital. Patients with TTP diagnosed between January 1996 and January 2005 were identified by a retrospective review of records of the Uludag University Hospital, Bursa (the largest referral center for adults with this disorder in this region with an estimated 2.2 million residents), which performs all therapeutic PE in the southern Marmara region in Turkey. A total of 11 (6 male, 5 female) patients were treated for TTP during this period. The median age was 39 years (range 18-49). One plasma volume exchange daily plus steroid was the principle treatment in all patients. A total of 295 PE sessions were performed. We have obtained six complete responses (CR) and three partial responses (PR) with daily PE and steroid (response rate 9/11). One of our primary refractory patients was saved with pulse steroid+cyclosporine+vincristine. Now, he is disease free for over one year. The other refractory patient did not develop any response to salvage therapy and expired on day 15 with status epilepticus and ventilator related pneumonia (mortality rate 1/11). A CR was obtained with adjuvant treatments in all three PR patients. Only one CR patient developed an early relapse (early relapse rate in CR patients 1/6). She was treated successfully with daily PE plus vincristine. Our median follow up period was 25 months (range 9-108). Considering our local population, our annual incidence is only about 0.63 new cases per one million people. This figure is considerably less than the data from US, which indicated an incidence of 3.7 cases per 1,000,000. To our knowledge, there is no high variability in the incidence of TTP in the different geographical regions of the world. It suggests that considerable number of patients escaped notice. We hope that, demonstrating the successful outcome, this article serves to urge primary physicians to keep in mind the diagnosis of TTP and refer suspected cases quickly.

译文

血栓性血小板减少性紫癜 (TTP) 是一种经典但不是血液学的常见疾病。等离子体交换 (PE) 显示几乎逆转其90% 死亡率。然而,这种戏剧性的疾病仍然有一些致命的结果。我们介绍了我们医院血浆置换加皮质类固醇治疗TTP的经验。通过对布尔萨乌鲁达格大学医院 (该地区最大的成人转诊中心,估计有220万名居民) 的记录进行回顾性审查,确定了1996年1月至2005年1月之间诊断出的TTP患者,该医院在土耳其南部马尔马拉地区进行所有治疗性PE。在此期间,共有11名 (6名男性,5名女性) 患者接受了TTP治疗。中位年龄为39岁 (18-49岁)。在所有患者中,每天一次血浆量交换加类固醇是主要治疗方法。总共进行了295次PE会话。我们已经获得了六个完全反应 (CR) 和三个部分反应 (PR) 与每日PE和类固醇 (反应率9/11)。我们的一名主要难治性患者被脉冲类固醇环孢素长春新碱挽救。现在,他一年多没有疾病。另一难治性患者对挽救治疗没有任何反应,并在第15天因癫痫持续状态和呼吸机相关性肺炎而死亡 (死亡率1/11)。所有三名PR患者均接受辅助治疗,获得CR。只有一名CR患者出现早期复发 (1/6 CR患者的早期复发率)。她接受了每日PE加长春新碱的治疗。我们的中位随访期为25个月 (范围9-108)。考虑到我们的当地人口,我们的年发病率仅为每100万人中0.63例新病例。这一数字大大低于美国的数据,后者表明每1,000,000有3.7例病例。据我们所知,在世界不同的地理区域,TTP的发病率没有很高的变异性。这表明相当多的患者逃脱了通知。我们希望,通过证明成功的结果,本文可以敦促初级医生牢记TTP的诊断并迅速转诊疑似病例。

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