Impaired B-cell differentiation and T-cell regulatory function in four patients with Bloom's syndrome
Clinical Immunology and ImmunopathologyPublished 1 February 1982
Noboru Taniguchi, Mikio Mukai, T Nagaoki, Toshio Miyawaki, N Moriya, Hiroaki Takahashi
Citations24
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TL;DR
It is suggested that B-cell differentiation ability and T-cell regulatory function for the generation of Ig-PC were incompletely developed in patients with Bloom's syndrome, as if functional maturation of the patients' lymphocytes had been arrested at the early developmental stages of immune ontogeny.
Abstract
Immunoglobulin-producing ability of lymphocytes from four patients with Bloom's syndrome was evaluated in the in vitro pokeweed mitogen (PWM)- and Nocardia water-soluble mitogen (NWSM)-driven culture systems. Serum levels of three major classes of immunoglobulins, IgG, IgM, and IgA, in patients were decreased as compared with age-matched controls, and the in vitro generation of immunoglobulin-producing cells (Ig-PC) was also impaired in all four patients, to an extent comparable with infant controls. Cellular defects participating in impaired generation of Ig-PC in one patient were assessed in the in vitro cross-over coculture system with autologous and allogeneic combinations of B and T cells and stimulation by PWM and NWSM. These experiments suggested that B-cell differentiation ability and T-cell regulatory function for the generation of Ig-PC were incompletely developed in these patients, as if functional maturation of the patients' lymphocytes had been arrested at the early developmental stages of immune ontogeny.
Keywords
Immunology and MicrobiologyMedicine
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