Abstract

Changes in cellular immunity have been reported in hemophiliacs receiving lyophilized factor concentrate, T cell subsets, mitogenic responsiveness, and degree of hypergammaglobulinemia in healthy hemophiliacs were examined in order to determine the effect of product therapy on immune responsiveness of children with hemophilia (age range, 3-21 years). The study population was comprised of 21 hemophilia A patients receiving factor VIII concentrate, 10 hemophilia A patients receiving cryoprecipitate, 8 hemophilia B patients receiving factor IX concentrate and 20 healthy children receiving no blood products. The percentage of T4+ lymphocytes was decreased in all hemophiliacs regardless of therapy (p<0.05). Only patients with hemophilia A receiving factor VIII concentrate exhibited a concomitant increase in the percentage of T8+ lymphocytes and showed a significantly decreased T4/T8 ratio (p<0.05). Depressed mitogenic responsiveness to phytohemagglutinin and concanavalin-A was observed in both hemophilia A and B patients receiving factor concentrate (p<0.05). No correlation was observed between changes in the T4/T8 ratio or mitogen responsiveness relative to the amount of product received. Hypergammaglobulinemia of the IgG class was demonstrable in all hemophilia groups and correlated with age (p<0.05). Phenotypic and functional alternations of lymphocytes demonstrated in hemophiliacs are probably the result of chronic factor exposure, which becomes more pronounced with increasing age.

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