Zenny J C, Chevrot A, Sultan Y, Godefroy D, Horreard P, Pallardy G
J Radiol. 1981 Apr;62(4):263-6.
Congenital afibrinemia is a rare disease, transmitted by an autosomal recessive mode, and due to deficient fibrinogen. Osseous involvement is exceptional, since only 2 cases have been reported. We describe a third case. The roentgenographic features are well limited radiolucent lesions, arising in the medullary space, and slightly expanding. These lesions are related to fibrous cysts, resulting from intraosseous hemorrhages. Spontaneous resolution may occur: the absence of fibrin deposits and blood clot probably favours resorption.
先天性无纤维蛋白原血症是一种罕见疾病，通过常染色体隐性模式遗传，病因是纤维蛋白原缺乏。骨骼受累情况罕见，因为仅报道过2例。我们在此描述第3例。X线特征为界限清晰的透亮病变，起源于骨髓腔且略有扩大。这些病变与骨内出血导致的纤维性囊肿有关。病变可能会自发消退：缺乏纤维蛋白沉积和血凝块可能有利于吸收。
