Table 1

Haematological and immunological blood values of the propositus

HematologyBirth1 year2 years2½ years3 years (BMT +5 days)5 years (BMT +2 years)
*Outside the normal range of values.
†Post-BMT transfusion values.
Hb (g/dL)15.99.5*2.0*6.8†13.1
Ht (L/L)0.530.330.07*0.40
Reticulocytes (106/mL)NT3.2<0.1*2.1
MCV (fL)NT907983.7
Platelets (106/mL)13415048320†379
Leukocytes (106/mL)18.99.41.1*0.18.1
% neutrophils68522252
% monocytes61086
% lymphocytes22356742
IgG11.59.65.211.3
IgA1.41.00.41.4
IgM1.00.70.3*1.3
Lymphocytes (106/mL)4.23.30.7*3.4
CD3+ T cells0.3*1.9
TcRαβ51%
TcRγδ49%
CD3+/4+ Th cells0.06*1.03
CD45RA7%81%
CD45RO84%22%
CD3+/8+ Tc cells0.1*0.74
CD45RA56%85%
CD45RO42%14%
CD19+ B cells0.21.11
CD16/56+ NK cells0.20.34