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Table 2 Evolution of the immune and autoimmune status of the proposita

From: Rituximab resistant evans syndrome and autoimmunity in Schimke immuno-osseous dysplasia

  Test results at the indicated ages
Age (years) 3§ 4 5
Test
(normal values)
   
IgG (g/l)
(5.53-10.20)
7.72 12.1 1.63
IgA (g/l)
(0.33-0.91)
0.83 1.66 0.43
IgM (g/l)
(0.47-1.67)
0.97 2.19 3.49
C3 (g/l)
(0.83-2.25)
1.06 1.47 1.41
C4 (g/l)
(0.14-0.35)
0.18 0.10 0.11
ANA negative negative negative
ANCA negative negative negative
ACLA (GPL/ml)
(0.8-11)
7.0 6.3 4.3
ENA screening negative negative NM
ATA IgA (U/ml)
(0-10)
2.09 2.11 NM
CD4 absolute count x103/ml (35-51%) 130 (12%) 200 (13%) 70 (50%)
CD8 absolute count x103/ml (18-40%) 20 (1.8%) 170 (11%) 60 (44%)
CD4/CD8
(1-3%)
6.7% 1.2% 1.1%
CD19 absolute count x103/ml (9-35%) 0.440 (45%) 530 (35%) 0 (0%)*
  1. §While receiving prednisone (60 mg/m2/day)
  2. Nephrotic syndrome worsened and proteinuria increased during the 5th year of life.
  3. *After rituximab treatment.