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Fetal B lymphocytes in mice and humans use a limited number of the available VH gene segments. Mouse fetal B cells primarily utilize 3' VH elements, suggesting that the localization of these elements determines their rearrangement frequency. The previously reported non-random usage of human VH genes has been more difficult to explain. In this study the authors analysed the expression of the most proximal 3' human VH element (VH6) using a monoclonal antibody (JE-6). VH6 expression was assessed in various B cell differentiation stages from fetal liver, bone marrow and spleen at 12-20 weeks of gestation. The authors demonstrate that the level of VH6 expression does not exceed a stochastic usage frequency. This suggests that the localization of VH6 does not significantly promote its expression during human fetal life, and that other factors must affect the usage of VH genes during human fetal development.

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An autosomal recessive type of severe combined immunodeficiency disease (SCID) was characterized by an absence of immunoglobulins (Ig) in the serum and of Ig+ lymphocytes in bone barrow (BM) and peripheral blood. In the BM CD10+/terminal deoxynucleotidyl transferase-positive lymphocytes were identified. Epstein-Barr virus-transformed B lymphoblastoid cell lines (BLCL) obtained from BM and peripheral blood did not synthesize Ig. The Ig heavy and light chain gene complexes in the BLCL had retained the germ-line configuration. Mature T cells were present but their numbers in peripheral blood were decreased. T lymphoblastoid cells derived from peripheral blood expressed normal T cell receptor (TcR) CD3 complexes and manifested various genomic TcR rearrangements. It was concluded that this type of SCID entailed a complete arrest of B lymphocyte differentiation in an early stage prior to Ig rearrangements and a quantitative defect of T lymphocytes which nevertheless allowed development of mature T cells. Repeated failures of BM transplantation and the striking absence of Ig assembly suggested that this SCID defect resides in the BM microenvironment.