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Cyclical shock with hyperglobulinemia.

A Larcan, M C Laprevote, H Lambert

    Bibliotheca Anatomica
    |January 1, 1975
    PubMed
    Summary
    This summary is machine-generated.

    This study describes a rare cyclical shock syndrome in adults, characterized by fluid shifts and IgG monoclonal gammopathy. The condition, potentially fatal, differs from multiple myeloma and may relate to periodic disease.

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    Area of Science:

    • Internal Medicine
    • Pathophysiology
    • Hematology

    Background:

    • A rare syndrome of cyclical shock, hemoconcentration, and hypoproteinemia is presented.
    • This syndrome occurs in adults without a known family history.
    • Monoclonal gammopathy is a key feature in affected individuals.

    Purpose of the Study:

    • To describe a distinct clinical syndrome characterized by recurrent shock episodes.
    • To investigate the underlying physiopathological mechanisms.
    • To differentiate this syndrome from other related conditions like multiple myeloma.

    Main Methods:

    • Comparative case analysis of three adult patients.
    • Clinical and laboratory data review, including assessment of monoclonal protein.

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  • Autopsy findings (where available) were analyzed.

    • Main Results:

    • Three adult cases exhibited cyclical shock with hemoconcentration and hypoproteinemia.
    • The underlying mechanism appears to be acute hyperpermeability.
    • All three cases showed IgG type monoclonal dysglobulinemia with light kappa chains; multiple myeloma was excluded.
    • Two of the three cases were fatal with negative autopsies.

    Conclusions:

    • A unique syndrome of cyclical shock associated with IgG monoclonal gammopathy in adults is identified.
    • Acute hyperpermeability is implicated as the core physiopathological process.
    • The syndrome warrants further comparison with periodic disease and primary amyloidosis for diagnostic clarity.