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[General variable immunologic deficiency with malabsorption syndrome].

A I Parfenov, N I Ekisenina, R B Gudkova

    Klinicheskaia Meditsina
    |May 1, 1990
    PubMed
    Summary

    General variable immunodeficiency (GVI) with malabsorption syndrome (MS) shows reduced immunoglobulin levels and T-lymphocytes. Gamma-globulin therapy offers some benefit but long-term outcomes remain challenging.

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    Area of Science:

    • Immunology
    • Gastroenterology
    • Clinical Medicine

    Context:

    • General Variable Immunodeficiency (GVI) is a primary immunodeficiency disorder.
    • Malabsorption Syndrome (MS) often complicates GVI, leading to significant clinical challenges.
    • This study focuses on a cohort of patients with co-existing GVI and MS.

    Purpose:

    • To evaluate the clinical course and immunological parameters in patients with GVI and MS.
    • To assess the efficacy of gamma-globulin replacement therapy in this patient population.
    • To determine long-term outcomes and mortality associated with GVI and MS.

    Summary:

    • Seventeen out of twenty-two GVI patients presented with severe MS.
    • Reduced serum immunoglobulins and T-lymphocyte counts were observed, with altered T-cell subpopulations.

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  • Elevated lymphokine production was noted in response to casein and milk albumin.
  • Adjuvant gamma-globulin therapy showed initial positive trends in clinical and immunological markers.
  • However, long-term follow-up revealed persistent partial compensation with recurrences in 15 patients.
  • Seven patients died due to pneumonia, cardiac failure, and visceral dystrophy.

    • Impact:

    • Highlights the critical need for GVI diagnosis in MS patients.
    • Recommends lifelong gamma-globulin replacement therapy for confirmed GVI cases.
    • Underscores the complex management and potential mortality associated with GVI and MS co-occurrence.