Ramanan A S, Thirumala S, Chandrasekaran V
Department of Pediatric Hematology/Oncology, Long Island Jewish Medical Center, New Hyde Park, New York, USA.
J Clin Apher. 1999;14(1):9-13. doi: 10.1002/(sici)1098-1101(1999)14:1<9::aid-jca2>3.0.co;2-3.
TTP is a disease with protean manifestations leading to errors in diagnosis. Critical reevaluation of a single observer's experience at LIJMC over a 7-year period is compared to that in published literature. We retrospectively analyzed presentation, clinical course, treatment, and outcome of 15 patients treated for TTP between 1990 and 1997 by one of the authors (V.C.). Minimal diagnostic criteria for TTP were unexplained moderate to severe thrombocytopenia (platelet count <100,000/cmm), microangiopathic hemolytic anemia, with or without low grade fever, and no other attributable etiologies. Neurologic and/or renal dysfunction constituted severe grade. Age range was 5-86 years, with one patient age 5, the youngest yet to date reported with classic TTP. Female to male ratio was 2:1. Overall survival rate was 87%; 40% of patients experienced immediate relapse within the first 4 weeks of presentation; and predisposing causes for immediate relapse appear to be intercurrent infections and severity of presentation. There was a 40% incidence of late relapses of TTP. Two patients with an unusually high number of late recurrences (6 and 16) were HCV-Ab positive and the possible role of persistent HCV infection in recurrent TTP was explored.
血栓性血小板减少性紫癜（TTP）是一种临床表现多样的疾病，容易导致诊断失误。将一位观察者在长岛犹太医疗中心（LIJMC）7年期间的经验与已发表文献中的经验进行批判性重新评估。我们回顾性分析了1990年至1997年间由作者之一（V.C.）治疗的15例TTP患者的临床表现、临床病程、治疗及转归。TTP的最低诊断标准为不明原因的中度至重度血小板减少（血小板计数<100,000/立方毫米）、微血管病性溶血性贫血，伴有或不伴有低热，且无其他可归因的病因。神经和/或肾功能障碍为重度。年龄范围为5至86岁，其中1例患者年仅5岁，是迄今为止报道的最年轻的经典TTP患者。男女比例为2:1。总生存率为87%；40%的患者在就诊后的前4周内立即复发；立即复发的诱发因素似乎是并发感染和病情严重程度。TTP的晚期复发率为40%。有2例患者晚期复发次数异常多（分别为6次和16次），丙型肝炎病毒抗体（HCV-Ab）呈阳性，因此探讨了持续性HCV感染在复发性TTP中的可能作用。
