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. Author manuscript; available in PMC: 2011 Jun 29.
Published in final edited form as: Br J Haematol. 2008 Nov 1;144(1):120–126. doi: 10.1111/j.1365-2141.2008.07416.x

Table I.

Haematological & immunological parameters in affected males

Case I.6 II.4 II.14 II.15 II.16 II.20 II.26 II.27 III.10b III.26b Normal adult range
Age (y) 45 32 34 33 32 23 18 16 8 7
Leucocytes a 1.7 5.1 2.8 4 2.3 1.7 2.1 3 4.6 5.2 4.0-11 * 109/L
Neutrophils 0.4 2.4 1.0 0.1 0.5 0.5 0.2 0.5 2.0 3.3 2.0-7.0 * 109/L
Monocytes 0.4 0.4 0.1 1.2 0.1 0.1 0.1 0.1 0.3 0.4 0.2-1.8 * 109/L
Eosinophils 0.0 0.1 0.2 0.1 0.0 0.0 0.2 0.2 0.1 0.1 0.02-0.5 * 109/L
Platelets 133 142 149 137 168 131 133 96 279 184 150-400 * 109/L
Lymphocytes 0.7 2.2 1.4 2.6 1.5 1.1 1.6 2.2 2.0 1.4 1.0-3.0 * 109/L
T-cells (CD3+) 690 1621 1153 2287 1418 881 1248 1861 1568 957 900-1700 * 106/L
 CD4+ 196 837 572 1339 752 517 636 955 946 513 700-1100 * 106/L
 CD8+ 493 782 646 938 620 337 676 804 692 456 500-900 * 106/L
 CD4/CD8 ratio 0.4 1.07 0.89 1.43 1.21 1.53 0.94 1.19 1.37 1.13 0.57-2.77
B-cells 75 422 169 410 150 210 268 317 562 304 200-400 * 106/L
NK-cells 23 50 39 35 48 21 21 55 42 32 200-400 * 106/L
IgA 0.56 1.32 1.06 1.8 0.92 0.59 0.53 1.49 0.89 0.29 0.80-2.80 g/L
a

data represent single random values, obtained on the same day for all cases. Values outside the normal range (2.5-97.5 % percentile) are bolded.

b

For the 2 paediatric cases, follow-up analyses are available in Supplementary Table 1 and 2.