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[Factor X deficiency and systemic amylosis].

作者信息

Pedinielli F J, Mascret B, Blanc A P, Sudan N, Juhan-Vague I, Carcassonne Y

出版信息

Sem Hop. 1984 Mar 8;60(11):792-4.

PMID:6324362
Abstract

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.

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