Table 2

The results of immunological evaluation in three patients with HDR syndrome

Patient (age at evaluation) A/II.1 (1.0 y) B/II.4 (3.5 y) C/II.1 (2.7 y)
T cells (%)72 (53.9–80.5)80 (41.8–77.8)73 (44.3–73.3)
B cells (%)25 (3.6–22.6)17 (1.7–16.8)23 (2.2–20.2)
Serum immunoglobulin levels (mg/dl)
 IgG995 (460–1220)926 (560–1390)1290 (530–1340)
 IgA32 (16–128)79 (31–202)172 (25–174)
 IgM65 (57–260)72 (66–288)223 (63–279)
Mitogen stimulation tests2-150 (cpm, 3H-thymidine uptake)
 PHA25 383 (26 000–53 0002-151)55 139 (26 000–53 0002-151)50 038 (26 000–53 0002-151)
 Con-A26 816 (20 000–48 0002-151)40 487 (20 000–48 0002-151)46 802 (20 000–48 0002-151)
Lymphocyte surface markers (%)
 CD4 (%)NE38.8 (23.7–57.3)30.0 (23.6–61.0)
 CD8 (%)NE33.1 (12.3–32.9)23.0 (8.8–30.8)
  • PHA, phytohaemagglutinin; Con-A, concanavalin A; CD, cellular (cluster) differentiation antigen; NE, not examined.

  • The values in parentheses except for those for mitogen stimulation tests represent age matched reference data.14 ,15

  • 2-150 Final concentration of a mitogen is 20 μg/ml for PHA and 7 μg/ml for Con-A.

  • 2-151 Reference values for normal adults.