When combined deficiency of both thymus - derived lymphocytes (T-cells) and bone-marrow-derived lymphocytes (B-cells) occurs as a congenital disorder, it is characterized by the onset in early life of severe viral, fungal, and bacterial infections and by early death, most often within the first two years of life.1 In congenital combined deficiency, there is a quantitative deficiency of both T-cells and B-cells. Combined T-cell and B-cell dysfunction may occur also in association with lymphoproliferative disorders. Observations on combined idiopathic late-onset immunoglobulin deficiency and severe qualitative T-cell dysfunction are rare.2 This paper describes the clinical courses and immunologic data in two patients with idiopathic late-onset combined immune deficiency.
A 51-year-old woman was first seen in January 1959. She reported having had fatigue, morning stiffness, and arthralgia of both hands, wrists, and elbows, recurrent respiratory infections, and splenomegaly for 12 years. The respiratory infections caused her to
Wilson WR, Hermans PE, Ritts RE. Idiopathic Late-Onset Immunoglobulin Deficiency With Functional T-Cell Deficiency. Arch Intern Med. 1976;136(3):343–346. doi:10.1001/archinte.1976.03630030073014
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