Idiopathic CD4+ T cell lymphocytopenia with the absence of B cells and CD8+28+ cells in peripheral blood

Clin Exp Med. 2002 Nov;2(3):143-6. doi: 10.1007/s102380200020.

Abstract

The absence of B cells and a severe decrease in CD8+28+ cells were observed in two female children with CD4+ T cell lymphocytopenia. Idiopathic (primary) CD4+ lymphocytopenia is a rare entity and its pathogenesis and genetics are not yet known. The literature was reviewed, in particular for severe alterations in B and CD8+28+ cells and for the role of NF-kappa B and p56 (lck) in the immunopathogenesis. Whether the underlying mechanism in idiopathic CD4+ lymphocytopenia is found or not, these patients who present with severe symptoms of a combined immunodeficiency must be treated with intravenous immunoglobin regularly until they have a compatible donor for bone marrow transplantation.

Publication types

  • Case Reports
  • Review

MeSH terms

  • B-Lymphocytes / immunology
  • CD28 Antigens / metabolism
  • CD4-Positive T-Lymphocytes / immunology*
  • CD8-Positive T-Lymphocytes / immunology
  • Child, Preschool
  • Female
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Lymphocyte Specific Protein Tyrosine Kinase p56(lck) / metabolism
  • Lymphopenia / immunology*
  • Lymphopenia / metabolism
  • Lymphopenia / therapy
  • NF-kappa B / metabolism
  • T-Lymphocyte Subsets / immunology

Substances

  • CD28 Antigens
  • Immunoglobulins, Intravenous
  • NF-kappa B
  • Lymphocyte Specific Protein Tyrosine Kinase p56(lck)