HEREDITARY-DEFICIENCY OF OKT4-POSITIVE CELLS - STUDIES FOR MODE OF INHERITANCE AND LYMPHOCYTE FUNCTIONS

Abstract

Two Graves'' patients were found to have no OKT4+ cells in their peripheral blood lymphocytes (PBL). However, the reactivity of their lymphocytes with T4(T4A) and anti Leu-3a was normal and no autoantibodies to the OKT4 determinant were found in their sera. Cytofluorographic analysis of PBL from their family members showed 3 types of immunofluorescence profiles with OKT4. The 1st type was the complete oKT4+ cell deficiency, the 2nd was the normal percentage of OKT4+ cells with half immunofluorescence intensity and the 3rd was the normal staining pattern with OKT4. Phytohemagglutinin (PHA) and pokeweed mitogen (PWM) induced blastogenesis; PWM-induced IgG synthesis, autologous and allogenic mixed lymphocyte reaction and interleukin-2 (IL-2) production of their PBL were also normal. Evidently, the expression of determinant to OKT4 is transmitted as autosomal incomplete dominant trait and OKT4+ cell deficiency is not accompanied by a lack of the inducer/helper subset of T cells.