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Cryoimmunoglobulinemia in four sisters.

F Dammacco, L Scarpioni, S Antonaci

    Acta Haematologica
    |January 1, 1978
    PubMed
    Summary
    This summary is machine-generated.

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    This study found mixed-typed cryoproteins (IgG and IgM) in four sisters, suggesting a genetic link in familial cryoglobulinemia. Two sisters had monoclonal IgM with chi light chains, associated with autoimmune symptoms.

    Area of Science:

    • Immunology
    • Genetics
    • Nephrology

    Background:

    • Cryoglobulinemia is characterized by the presence of cryoglobulins in the blood, which can lead to various clinical manifestations.
    • Mixed-type cryoglobulinemia involves immune complexes composed of IgG and IgM antibodies.

    Observation:

    • Four sisters presented with mixed-typed cryoproteins (IgG and IgM) in their sera.
    • Two sisters exhibited monoclonal IgM with kappa light chains, alongside polyclonal IgG.
    • Clinical diagnoses varied, including purpura-weakness-arthralgias syndrome, posthepatitis cirrhosis, congestive heart failure, and mitral stenosis.

    Findings:

    • Cryocrit levels ranged from 3% to 16% among the sisters.
    • Rheumatoid factor activity was consistently detected in cryoprecipitates and isolated IgM.

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  • Renal biopsy revealed endomembranous deposits of IgG and IgM in one patient.

    • Implications:

    • The familial occurrence suggests a potential genetic predisposition, possibly autosomal recessive.
    • This case highlights the diverse clinical spectrum of cryoglobulinemia.
    • Understanding the genetic basis can aid in diagnosing and managing familial cryoglobulinemia.