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Systemic amyloidosis associated with factor X deficiency.

H Shibuya, N Azumi, F Abe

    Acta Pathologica Japonica
    |May 1, 1984
    PubMed
    Summary
    This summary is machine-generated.

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    This study reports a rare case of amyloidosis linked to factor X deficiency. Rapid amyloid deposition, particularly in the liver and spleen, likely caused the severe factor X deficiency observed.

    Area of Science:

    • Hematology
    • Pathology
    • Medical Science

    Background:

    • Amyloidosis is a group of diseases characterized by the deposition of amyloid proteins in various organs.
    • Factor X deficiency is a rare bleeding disorder caused by insufficient levels of coagulation factor X.

    Observation:

    • A patient presented with markedly decreased factor X levels (9% normal) and a shortened half-life of infused factor X.
    • Autopsy revealed extensive amyloid deposition in visceral organs, notably the liver and spleen.
    • Amyloid was identified as AL protein, resistant to potassium permanganate, with Bence Jones protein detected post-therapy.

    Findings:

    • Electron microscopy showed amyloid fibrils originating from Kupffer cell membranes, suggesting rapid amyloid turnover.
    • The severity and rapidity of amyloid deposition correlated with the observed factor X deficiency.

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    Implications:

    • This case highlights a potential pathogenic link between systemic amyloidosis and acquired factor X deficiency.
    • Understanding this association may inform diagnostic and therapeutic strategies for patients with both conditions.