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血栓性血小板减少性紫癜(TTP)在儿童病例中甚为少见,但如未能及时诊断及施予治疗,其后果则极为严重。其最常见之5种病症为:血小板减少、微血管溶血性贫血、急性肾衰竭、发热及中枢神经系统症状。但临床病例中,并不一定会同时出现上述5种症状。故此医疗人员对此病必须有极高之警觉性。TTP之病理特征包括:外周血涂片可见裂体细胞,Coombs试验阴性,血清乳酸脱氢酶增高及中度或重度血小板减少。TTP发病机理主因缺乏ADAMTS13,从而引发微血管溶血性贫血及血小板减少。TTP可概括分为家族性TTP(Upshaw Schulman综合征)和继发性TTP。家族性TTP是由于先天性ADAMTS13缺乏所致,其急性治疗法为血浆置换,当病情稳定后,可输注新鲜冰冻血浆以防止病情复发。继发性TTP是指患者因体内产生抗体而导致ADAMTS13功能减退,主要治疗方法亦为血浆置换,最新之临床文献显示rituxiamb对此症亦颇有治疗价值。
Thrombotic thrombocytopenic purpura (TTP) is rare in childhood cases, but if it is not diagnosed and treated in time, the consequences are extremely serious. The five most common conditions are: thrombocytopenia, microvascular hemolytic anemia, acute renal failure, fever and central nervous system symptoms. However, in clinical cases, the above five symptoms do not always appear at the same time. Therefore, the medical staff must be extremely alert to the disease. TTP pathological features include: peripheral blood smear cells can be seen split, Coombs test negative, elevated serum lactate dehydrogenase and moderate or severe thrombocytopenia. The main pathogenesis of TTP is the lack of ADAMTS13, triggering microvascular hemolytic anemia and thrombocytopenia. TTP can be broadly divided into familial TTP (Upshaw Schulman syndrome) and secondary TTP. Familial TTP is due to the lack of congenital ADAMTS13, its acute treatment of plasma exchange, when the disease is stable, the infusion of fresh frozen plasma to prevent the recurrence of the disease. Secondary TTP refers to patients with antibody production in vivo resulting in ADAMTS13 dysfunction, the main treatment is also plasma exchange, the latest clinical literature shows that rituxiamb also has considerable therapeutic value.