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Progressive change in lymphocyte distribution and degree of hypergammaglobulinemia with age in children with hemophilia

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Abstract

Fifty asymptomatic, pediatric hemophiliacs were examined for distribution of T-cell subsets, responsiveness to mitogen stimulation, interleukin-2 production, hypergammaglobulinemia, and the presence of antibody to virus including the human T-lymphotrophic virus type III (HTLV-III). Hemophilia A patients receiving factor VIII concentrate as replacement therapy had the most pronounced changes including decreased T4/T8 ratios and lowerin vitro responsiveness to both phytohemagglutinin and pokeweed mitogen. Hemophilia A patients treated with cryoprecipitate and hemophilia B patients did not demonstrate these changes. Regardless of replacement therapy, hemophiliacs demonstrated a progressive decrease in the T4/T8 ratio and a progressive increase in the degree of IgG hypergammaglobulinemia as they aged. The amount of factor or cryoprecipitate or exposure to virus did not influence the T4/T8 ratio. These changes appear to be a result of chronic product exposure, which becomes more pronounced with increasing age.

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Shannon, B.T., Roach, J., Cheek-luten, M. et al. Progressive change in lymphocyte distribution and degree of hypergammaglobulinemia with age in children with hemophilia. J Clin Immunol 6, 121–129 (1986). https://doi.org/10.1007/BF00918744

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