Hereditary CD4+ T lymphocytopenia

Abstract

Two siblings suVering from mental retardation, progressive bronchiectasis, extensive warts, and persistent hepatitis B are described. The propositus also had an unusual physiognomy and non-specific colitis. Both patients had a marked decrease in the population of CD4+ helper T cells.

Lymphocyte Diversity in a 9-Year-Old Boy with Idiopathic CD4+ T Cell Lymphocytopenia

“…The normal density of CD3 expression of T cells found in our patient may be explained by the peripheral expansion of T cells. Other reports about ICL show a great variability in T cell subsets, ranging from normal to enhanced expression of CD45RO [23, 24, 25]. Depletion of CD4+CD45RA+ cells could be caused by accelerated apoptosis of these cells, but we found that the apoptotic rate of CD4+CD45RA+ cells was not increased compared to healthy controls.…”

Promoter IV of the class II transactivator gene is essential for positive selection of CD4+ T cells

“…Recently, 2 cases of hereditary CD4 ϩ T-cell lymphopenia with normal serum levels were also reported. 81 Interestingly both patients presented with extensive warts. Epidermodysplasia verruciformis (EV) is a heritable syndrome that is also characterized by extensive warts and is often associated with CD4 ϩ T-cell lymphopenia.…”

Idiopathic CD4+ T-lymphocytopenia in a boy with Down syndrome. Report of a patient and a review of the literature

“…Patients with Down syndrome are known to have immunological abnormalities including decreased numbers of CD4+ T-lymphocytes [5], but the extent of CD4+ T-lymphocytopenia is milder than that observed in ICL [1]. Genetic factors may cause ICL [2,6], and the identification of trisomy 21 as a cause of ICL may aid in the identification of these genes. …”