Bleeding and thrombosis in 55 patients with inherited afibrinogenaemia
Bleeding and thrombosis in 55 patients with inherited afibrinogenaemia
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55例遗传性无纤维蛋白原血症患者出血及血栓形成
DOI：
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发表时间：
1999
影响因子：
6.5
通讯作者：
P. Mannucci
中科院分区：
文献类型：
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作者：
M. Lak;M. Keihani;F. Elahi;F. Peyvandi;P. Mannucci
遗传性无纤维蛋白原血症患者的症状谱的知识是有限的罕见的凝血缺陷。我们比较了来自伊朗的55名无纤维蛋白原血症患者与100名严重因子VIII缺乏症患者的大系列研究。在无纤维蛋白原血症中，粘膜型出血症状的频率较高，但关节和肌肉出血的频率和严重程度低于血友病。脐带出血仅在无纤维蛋白原血症患者中相对常见。2例年轻患者发生自发性血栓形成，3例女性发生复发性流产。总的来说，无纤维蛋白原血症的出血症状在性质上是不同的，比血友病严重。无纤维蛋白原血症也可伴有血栓形成表现。
Knowledge of the spectrum of symptoms in patients with inherited afibrinogenaemia is limited by the rarity of this coagulation defect. We compared a large series of 55 afibrinogenaemic patients from Iran with 100 patients with severe factor VIII deficiency. In afibrinogenaemia there was a higher frequency of mucosal‐type bleeding symptoms but joint and muscle bleeding was less frequent and severe than in haemophilia. Umbilical cord bleeding was relatively frequent only in afibrinogenaemic patients. Two young patients developed spontaneous thrombotic episodes and three women had recurrent abortions. Overall, in afibrinogenaemia bleeding symptoms are qualitatively different and less severe than in haemophilia. Afibrinogenaemia can also be accompanied by thrombotic manifestations.