McPherson R A, Onstad J W, Ugoretz R J, Wolf P L
Am J Hematol. 1977;3:225-35. doi: 10.1002/ajh.2830030303.
Combined severe deficiencies of blood clotting factors IX and X were observed in 2 patients who suffered from systemic amyloidosis. This unique deficiency state was marked by refractoriness to Vitamin K as well as to transfusion therapy. Increased antithrombin activity was present in both individuals and corresponded in time to the emergence of a monoclonal IgG kappa light chain paraprotein in 1. Both patients demonstrated profound bleeding disorders. It is hypothesized that the Vitamin K dependent factors have special affinity for amyloid deposits due to an unusual amino acid (gamma-carboxyglutamic acid) present in these factors.
在2例患有系统性淀粉样变性的患者中观察到凝血因子IX和X联合严重缺乏。这种独特的缺乏状态的特征是对维生素K以及输血治疗均无反应。两名患者的抗凝血酶活性均升高，且其中1例患者的抗凝血酶活性升高时间与单克隆IgG κ轻链副蛋白的出现相一致。两名患者均表现出严重的出血性疾病。据推测，由于这些因子中存在一种不寻常的氨基酸（γ-羧基谷氨酸），维生素K依赖因子对淀粉样沉积物具有特殊亲和力。
