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[Factor X deficiency and systemic amylosis].

F J Pedinielli, B Mascret, A P Blanc

    La Semaine Des Hopitaux : Organe Fonde Par L'Association D'Enseignement Medical Des Hopitaux De Paris
    |March 8, 1984
    PubMed
    Summary
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    Systemic amyloidosis can cause severe factor Stuart deficiency, leading to hemorrhagic syndrome. Amyloid fiber affinity for factor Stuart may explain this rare complication and the effectiveness of splenectomy.

    Area of Science:

    • Hematology
    • Pathology
    • Biochemistry

    Background:

    • Systemic amyloidosis is a rare condition characterized by amyloid protein deposition in organs.
    • Hemorrhagic syndromes can be a severe manifestation of systemic amyloidosis.
    • Factor Stuart (coagulation factor X) deficiency is an uncommon but significant bleeding disorder.

    Observation:

    • A 52-year-old patient presented with a major hemorrhagic syndrome.
    • Diagnosis of systemic amyloidosis was confirmed via osteomedullary biopsy.
    • The hemorrhagic syndrome was attributed to severe factor Stuart deficiency.

    Findings:

    • The study highlights the link between systemic amyloidosis and factor Stuart deficiency.
    • Amyloid classification based on biochemical and immunological data is reviewed.

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  • Plasmocyte-macrophage cooperation is implicated in amyloid pathogenesis.

    • Implications:

    • Amyloid fiber affinity for factor Stuart and "mass effect" may cause deficiency.
    • Understanding this mechanism could refine treatment strategies for amyloidosis-related bleeding.
    • Splenectomy's efficacy in select cases may be explained by amyloid load reduction.