Pedinielli F J, Mascret B, Blanc A P, Sudan N, Juhan-Vague I, Carcassonne Y
Sem Hop. 1984 Mar 8;60(11):792-4.
The case of a 52-year-old patient with systemic amylosis diagnosed upon examination of an osteomedullary biopsy specimen is reported. The most prominent clinical manifestation was a major hemorrhagic syndrome which was recognized as being a result of severe factor Stuart deficiency. The new classification of amyloses based on the latest biochemical and immunological findings is recalled. Current physiopathogenic speculations put emphasis on plasmocyte-macrophage cooperation. Lastly, factor Stuart deficiency, which is as classical as it is uncommon, could originate in the particular affinity of amyloid fibers for this factor and in a "mass effect" (quantity of amyloid substance directly exposed to blood flow), explaining why splenectomy is effective in some instances.
报告了一例52岁的患者，经骨髓活检标本检查诊断为系统性淀粉样变性。最突出的临床表现是严重的出血综合征，被认为是由于严重的斯图尔特因子缺乏所致。回顾了基于最新生化和免疫学发现的淀粉样变性新分类。目前的病理生理推测强调浆细胞-巨噬细胞的协同作用。最后，斯图尔特因子缺乏虽典型但不常见，可能源于淀粉样纤维对该因子的特殊亲和力以及“质量效应”（直接暴露于血流中的淀粉样物质数量），这解释了为何脾切除术在某些情况下有效。
