Laboratory characteristics

The hallmarks of XLA are markedly decreased concentrations of serum immunoglobulins and an almost complete absence of B cells in the peripheral circulation. The serum immunoglobulin G (IgG) is usually less than 2mgml_l, and the IgM and IgA are less than 0.1 mgml-'. B cells, which constitute 5-18% of the peripheral blood lymphocytes in the normal individual, cannot be detected using standard techniques; however, a small number (0.005-0.1% of peripheral blood lymphocytes) are uniformly present. As evaluated with antibodies to surface markers, these B cells have an immature phenotype that is similar to that seen in B cells from X-linked immunodeficient (xid) mice. Epstein-Barr virus (EBV)-transformed B cell lines, produced from the blood of patients with XLA, make normal immunoglobulins with correct rearrangements of V, D and J gene segments. A disproportionate number of the pre-B cells found in the bone marrow of patients with XLA are at the earliest stage of development and a reduced percentage of them are in the S phase of the cell cycle.

Abnormalities in cell lineages other than B cells have been reported; however, these abnormalities may be secondary to the marked reduction in B cell numbers or the hypogammaglobulinemia rather than a direct effect of the genetic defect.

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