Nishino M, Nishino S, Sugimoto M, Shibata M, Tsuji S, Yoshioka A
Department of Pediatrics, Nara Prefectural Mimuro Hospital, Japan.
Int J Hematol. 1996 Aug;64(2):127-34. doi: 10.1016/0925-5710(96)00470-7.
The changes of the FVIII binding capacity of vWF after the infusion of FVIII/vWF concentrate was studied in two patients with type 2N vWD, and also during pregnancy in one of them. After infusion of FVIII or DDAVP to the patients, FVIII:C in plasma increased as expected, but it then decreased, with a markedly short half-life, of about 2 h, due to the defect in the FVIII binding capacity of vWF in plasma. However, after infusion of FVIII/vWF concentrate (40 U of FVIII:C/kg), FVIII:C increased, from 4-6 to 100-160 U/dl, more than the expected values, and decreased with the half-life expected. The FVIII binding capacity of vWF in plasma changed in parallel with the concentration of exogenous normal vWF, with a half-life of more than 24 h. During pregnancy, no increase of FVIII:C was observed, although vWF:Ag increased from 40 (before pregnancy) to 90 U/dl in plasma at 35th week of gestation. The FVIII binding capacity of vWF in plasma showed no increase during pregnancy. Accordingly, the administration of FVIII/vWF concentrate to the patient at delivery resulted in adequate hemostasis.
在两名2N型血管性血友病（vWD）患者中研究了输注FVIII/vWF浓缩物后vWF的FVIII结合能力变化，其中一名患者还在孕期进行了研究。给患者输注FVIII或去氨加压素（DDAVP）后，血浆中的FVIII:C如预期那样升高，但随后下降，半衰期明显较短，约为2小时，这是由于血浆中vWF的FVIII结合能力存在缺陷。然而，输注FVIII/vWF浓缩物（40 U FVIII:C/kg）后，FVIII:C从4 - 6 U/dl升高至100 - 160 U/dl，高于预期值，并以预期的半衰期下降。血浆中vWF的FVIII结合能力与外源性正常vWF的浓度平行变化，半衰期超过24小时。孕期未观察到FVIII:C升高，尽管在妊娠第35周时血浆中vWF:Ag从妊娠前的40 U/dl升高至90 U/dl。血浆中vWF的FVIII结合能力在孕期未升高。因此，在分娩时给该患者输注FVIII/vWF浓缩物可实现充分止血。
