Table I.

Phenotype of patients

CharacteristicP1P2aP3aP4P5Age-matched controls
Age at diagnosis (yr)66844
Serum Ig levels (g/l)
    IgM164.12.62.80.90.5–1.2
    IgG0.63.65.70.71.46.8–12.5
    IgA0.3<0.03<0.03<0.03<0.030.6–1.6
T lymphocyte counts/μl2,4001,4201,2381,6121,3001,200–2,600
B lymphocyte counts/μl452120170462440110–570
IgD+/CD19+ (%)b99.999.2ND99.8ND83–93
CD27+/CD19+ (%)b1.53.33.14.73.113–58
  • a P2 and P3 are siblings.

  • b Gated on CD19+ B cells.