Publication | Open Access
Selective IgM deficiency in adults: phenotypically and functionally altered profiles of peripheral blood lymphocytes.
46
Citations
30
References
1987
Year
Clinical ImmunologyImmunodeficienciesImmunologyPathologyImmune SystemImmunotherapyImmunogeneticsPeripheral Blood LymphocytesHematologyAllergyAutoimmune DiseaseAutoimmunityHumoral ImmunityImmunologic DiseaseSelective Igm DeficiencyInborn Error Of ImmunityB Cell SubpopulationsImmune Cell DevelopmentSurface ImmunoglobulinsMedicine
Peripheral blood lymphocytes from four patients with selective IgM deficiency were examined phenotypically and functionally. Although B cell subpopulations determined by surface immunoglobulins were within normal or nearly normal range, T8+ cells were significantly increased and T4/T8 ratios were inverted in three patients. IgM specific hyporesponsiveness in the PWM-driven immunoglobulin production system was observed in all four patients. Ia-like antigen positive T cells were increased in two patients; both had increased Leu2a+ Leu15+ suppressor-effector cells. In addition, Leu3a+ Leu8+ suppressor-inducer cells were increased in one of these two patients. Excessive (either IgM-specific or isotype non-specific) suppressor activity of T cells and IgM specific hyporesponsiveness of non-T cells were observed in these two patients in the recombination plaque assay. Although these results showed the complexity of the pathogenesis of this syndrome, they suggested that suppressor-associated T cells may play a role in some patients with selective IgM deficiency.
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