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Table 1 Immunological repertoire obtained prior to the onset of anti-IL1B therapy evidencing multiple immune defects (relevant alterations shown in bold)

From: Immunological repertoire linked to PSTPIP1-associated myeloid-related inflammatory (PAMI) syndrome

Immunological Profile

Index Patient Before anti-IL1 (%)

Age Matched Reference Range

Mother untreated (%)

Age Matched Reference Range

CD3+

38,9

66.2 (57.1-72.7)

81

75.1 56.8-84.1

CD4+

22,6

37.7 (27.7-46.3)

36,5

40.7 26.9-55.5

CD8+

70,7

21.9 (15.7-33.8)

58

27.2 13.3-41.5

CD4+ naive

23,9

70.30 (46.14-84.40)

16,6

32,7 (20,9-49,1)

CD4+ TCM

10,8

26.40 (13.88-48.12)

32

33 (20,8-45,6)

CD4+ TEM

5,41

2,8 (0.94-6.46)

11,4

25,1 (13,9-33,6)

CD4+ TEMRA

1,59

0.2 (0.00-1.36)

1,4

4,9 (2-10,3)

CD19+

21,3

19,3 (13.3-26.7)

9,16

12 (5.9-20.6)

CD3-CD16+CD56+(NK)

1,2

7,3 (7,8-16,1)

3,34

8.2 (2.8-24.1)

CD3+HLA-DR+

43,5

NR

48,7

NR

CD20+CD27+(LB mem)

27,9

NR

29,1

NR

CD3+TCRabCD4-CD8-

2,83

Total Lymphocytes (<1,5%)

2,01

Total Lymphocytes (<1,5%)

 

7,54

CD3+(<2,5%)

NM

CD3+(<2,5%)

CD3+B220+

31,7

Total Lymphocytes (NR)

15,8

Total Lymphocytes (NR)