Mazzucconi M G, Solinas S, Chistolini A, Motta M, Mariani G
Nouv Rev Fr Hematol (1978). 1985;27(5):303-5.
A case of severe factor V deficiency that developed an inhibitor to factor V following the treatment with fresh frozen plasma (FFP) is described. The patient had a CRM-negative form of factor V congenital deficiency: no factor V antigen could be assayed in her plasma. A sister who supposedly had a severe factor V deficiency died as a result of a severe posttransfusional hepatitis. Two other members of the family were found to be heterozygotes. After several exposures to FFP, the propositus became refractory to the treatment with the appearance of a low titre inhibitor (5 units) which, nevertheless, could not be overcome by plasma infusions.
本文描述了一例严重的凝血因子V缺乏症患者，在接受新鲜冰冻血浆（FFP）治疗后产生了针对凝血因子V的抑制物。该患者患有CRM阴性型先天性凝血因子V缺乏症：其血浆中无法检测到凝血因子V抗原。据推测患有严重凝血因子V缺乏症的一名姐妹因严重的输血后肝炎死亡。该家族的另外两名成员被发现为杂合子。在多次接触FFP后，先证者对治疗产生了耐药性，出现了低滴度抑制物（5单位），然而，血浆输注无法克服这种抑制物。
