Novel T-Lymphocyte Population in Combined Immunodeficiency with Features of Graft-versus-Host Disease

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Abstract
Wirt and collegues (Aug. 10 issue)1 described a very interesting patient who had a new subpopulation of T cells expressing the alpha/beta form of the T-cell receptor (TCRα/β) but neither CD4 nor CD8. They suggested that the proliferation of the novel T-cell population might be due to aberrant T-cell differentiation. However, except for the T-cell phenotype, their patient had many features similar to those of patients with granular lymphocyte proliferative disorders,2 which are occasionally associated with various infections. The T cells of patients who have these disorders often have cytotoxic and suppressor function and are infiltrative, resulting in anemia, thrombocytopenia, hepatosplenomegaly, skin lesions, and hypogammaglobulinemia,3 , 4 which were observed in their patient. We recently encountered several pediatric patients with immunodeficiency and granular lymphocyte proliferative disorders associated with Epstein–Barrvirus infection.