Abnormal Structure of von Willebrand Factor in Myeloproliferative Syndrome Is Associated to Either Thrombotic or Bleeding Diathesis
Abnormal Structure of von Willebrand Factor in Myeloproliferative Syndrome Is Associated to Either Thrombotic or Bleeding Diathesis
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骨髓增殖综合征中冯维勒布兰德因子的异常结构与血栓或出血素质有关
DOI：
10.1055/s-0038-1645964
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发表时间：
1987
影响因子：
6.7
通讯作者：
J. Batlle
中科院分区：
文献类型：
--
作者：
M. López;C. López;R. Martín;A. Pardo;F. Ramos;J. Batlle
摘要 对 8 名骨髓增殖综合征 (MS) 患者的血浆冯维勒布兰德因子 (vWF) 的多聚体和亚基模式进行了分析，以研究先前在该实体中观察到的蛋白质“体内”蛋白水解裂解中可能存在的异质性。六名患者缺乏大的 vWF 多聚体，其中五名患者具有正常的出血时间 (BT) 和临床记录的血栓起源发作，而一名患者具有长 BT 和出血症状。 7 名患者的 176 kDa 亚基片段相对增加，而只有 1 名患者的 189 kDa 多肽增加。此外，另一名患者（在任何治疗之前）显示存在约 95 kDa 的新片段，该片段在白消安治疗后消失。从这些服用蛋白酶抑制剂的患者身上采集血液并没有纠正异常情况。两名 vWF 异常患者输注 DDAVP 时，伴随着： 出现较大的 vWF 多聚体，并迅速从血浆中消失；每个多聚体卫星带相对比例的增加以及176 kDa片段的进一步增加。这些数据表明 MS 中存在的 vWF 异常存在一些异质性，这可能部分与“体内”而不是“体外”发生的 vWF 蛋白水解程度不同有关，并且可能与血栓形成或出血素质有关。他们还表明，尽管存在异常的、已经蛋白水解的 vWF，但 DDAVP 增强的蛋白水解在 MS 中发生，其程度与正常个体中描述的相似。
Summary The multimeric and subunit patterns of plasma von Willebrand factor (vWF) were analyzed in eight patients with myeloproliferative syndrome (MS) in order to investigate the possible existence of heterogeneity in the “in vivo” proteolytic cleavage of the protein, previously observed in this entity. Six patients lacked large vWF multimers, five of them having normal bleeding times (BT) and clinically documented episodes of thrombotic origin, whereas one patient had long BT and bleeding symptoms. Seven patients showed a relative increase in the 176 kDa subunit fragment while the 189 kDa polypeptide was increased in only one. In addition, another patient (and prior to any therapy) showed the presence of a new fragment of approximately 95 kDa which disappeared after Busulfan therapy. The collection of blood from these patients with proteinase inhibitors did not correct the abnormalities. The infusion of DDAVP to two patients with abnormal vWF was accompanied by: the appearance of larger vWF multimers which disappeared rapidly from plasma; an increase in the relative proportion of the satellite bands of each multimer and a further increase of the 176 kDa fragment. These data point to some heterogeneity in the vWF abnormality present in MS which may be related in part to a variable degree of proteolysis of vWF occurring “in vivo” rather than “in vitro”, and which may be associated to either a thrombotic or a bleeding diathesis. They also suggest that despite the presence of abnormal, already proteolyzed vWF, DDAVP-enhanced proteolysis occurs in MS to a similar extent to what is described in normal individuals.
DOI：
10.1172/jci112394
发表时间：
1986
期刊：
The Journal of clinical investigation
影响因子：
--
作者：
Zimmerman,TS;Dent,JA;Ruggeri,ZM;Nannini,LH
通讯作者：
Nannini,LH
骨髓增殖综合征患者血浆冯维勒布兰德因子的亚基组成。
影响因子：
20.3
作者：
Budde,U;Dent,JA;Berkowitz,SD;Ruggeri,ZM;Zimmerman,TS
通讯作者：
Zimmerman,TS