Deroux A, Sirodot M, Daguindau N, Barro C, Coppo P, Bouillet L
Service de médecine interne, hôpital Michallon, CHU de Grenoble, boulevard de la Chantourne, 38700 La Tronche, France.
Service de réanimation polyvalente, centre hospitalier d'Annecy, 74000 Annecy, France.
Rev Med Interne. 2016 Jan;37(1):13-8. doi: 10.1016/j.revmed.2015.05.003. Epub 2015 Jun 9.
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy due to platelet microthrombosis involved in multiple systems associated with multiple organ dysfunctions and often severe disease course.
In order to enhance the understanding of TTP, the clinical features, laboratory characteristics, treatment and outcome of 27 patients with TTP were retrospectively analyzed and investigated in two centres (Annecy and Grenoble).
All the 27 patients presented with haemolytic anemia and decreased platelet counts. Eight patients had fever. Thirteen patients had kidney involvement including proteinuria and renal function abnormalities. Eighteen patients had neurological manifestations. Association of the 5 characteristic features of TTP was rarely found (11%) which could lead to diagnosis delay. The von Willebrand factor-cleaving protease (ADAMTS13) activity was less than 10% in all patients. At the same time, plasma ADAMTS13 inhibitors were detected in 20 patients out of the 27. After treatment with plasma exchange, glucocorticoid and rituximab, 23 patients (85%) achieved complete remission. Four patients died, in which two cases were secondary to late diagnosis.
TTP is a thrombotic microangiopathy often associated with multiple organ dysfunctions. Salvage therapy, based on some studies experiences and discussed with the Reference Center, could help getting answers in most severe cases. Awareness of physicians is an important step to further limit any delay in medical care, and to further improve the prognosis of patients with TTP.
血栓性血小板减少性紫癜（TTP）是一种血栓性微血管病，由血小板微血栓形成所致，累及多个系统，伴有多器官功能障碍，疾病进程通常较为严重。
为加深对TTP的了解，对两个中心（阿讷西和格勒诺布尔）的27例TTP患者的临床特征、实验室检查特点、治疗及转归进行回顾性分析和研究。
27例患者均有溶血性贫血和血小板计数减少。8例患者发热。13例患者有肾脏受累，包括蛋白尿和肾功能异常。18例患者有神经学表现。TTP的5个特征性表现很少同时出现（11%），这可能导致诊断延迟。所有患者的血管性血友病因子裂解蛋白酶（ADAMTS13）活性均低于10%。同时，27例患者中有20例检测到血浆ADAMTS13抑制剂。经过血浆置换、糖皮质激素和利妥昔单抗治疗后，23例患者（85%）实现完全缓解。4例患者死亡，其中2例死于诊断延迟。
TTP是一种常与多器官功能障碍相关的血栓性微血管病。基于一些研究经验并与参考中心讨论的挽救治疗，有助于在大多数严重病例中找到解决方案。医生的认识是进一步减少医疗延误、改善TTP患者预后的重要一步。
