Barbot J, Costa E, Guerra M, Barreirinho M S, Isvarlal P, Robles R, Gerritsen H E, Lämmle B, Furlan M
Serviço de Hematologia do Hospital de Crianças Maria Pia, Porto, Portugal.
Br J Haematol. 2001 Jun;113(3):649-51. doi: 10.1046/j.1365-2141.2001.02808.x.
We report the results of 10 years of prophylactic fresh-frozen plasma (FFP) infusion therapy in a 14-year-old girl with chronic relapsing thrombotic thrombocytopenic purpura (TTP), in whom a severe congenital von Willebrand factor (VWF)-cleaving protease deficiency has been documented. Severe haemolytic crises triggered by infections were prevented and her present renal and neurological functions have been preserved. Sequential measurements of protease activity and platelet count after FFP infusion led us to conclude tentatively that 5% may be sufficient to degrade very large and adhesive VWF multimers.
我们报告了对一名14岁慢性复发性血栓性血小板减少性紫癜（TTP）女孩进行10年预防性新鲜冷冻血浆（FFP）输注治疗的结果，该女孩已被证明存在严重的先天性血管性血友病因子（VWF）裂解蛋白酶缺乏。感染引发的严重溶血危机得到了预防，她目前的肾脏和神经功能得以保留。FFP输注后对蛋白酶活性和血小板计数的连续测量使我们初步得出结论，5%的FFP可能足以降解非常大的粘附性VWF多聚体。
