A case of common variable immunodeficiency with defect of helper T-cell function

Abstract

A 20 year-old female patient with recurrent upper respiratory infection was found to have hypogammaglobulinemia (0.08g/dl) and low serum levels of whole immunoglobulin classes (IgG: 182mg/dl, IgA: less than 0.7mg/dl, IgM: 5.0mg/dl, IgD: less than 2.0mg/dl, IgE: 0.2u/ml) under the diagnosis of common variable immunodeficiency. She had leucopenia of WBC: 3, 600/cumm, Ly: 35%, and T-cell: 85%, B-cell: 12%, OKT3+: 73.1%, OKT4+: 26.6%, OKT8+: 40.3%, OKT 4/8 ratio: 0.66, in association with hypochromic anemia of RBC: 433×104/cumm, Hb: 9.9g/dl, s-Fe: 43μg/dl, UIBC: 367μg/dl.In vitro IgG synthesis by peripheral blood mononuclear cells from this patient in culture with stimulation of Pokeweed Mitogen (10μl/ml) was lower than controls. The amount of IgG produced by B-cells from the patient was enhanced in co-culture with T-cells from normal donors. T-cells from the patient could not increase IgG synthesis by normal B-cells in coculture. And the addition of patient T-cells as the third population into the culture of normal T- and B-cells did not suppress IgG production. These results indicated that hypogammaglobulinemia found in this patient was caused by defect of helper T-cell function rather than abnormality of B-cells in response to help of normal T-cells or enhanced suppressor T-cell activity.The co-existence of helper T-cell dysfunction and iron deficiency anemia found in this patient is intriguing with speculation that lymphoid systems play a role in iron absorption through small intestine.