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Table 3 Absolute counts and frequencies of CD4+ T cell, CD8+ T cell, and CD4+ Treg cell subsets in the peripheral blood of healthy controls and PD patients

From: Prolidase deficiency, a rare inborn error of immunity, clinical phenotypes, immunological features, and proposed treatments in twins

Cell type

Subset

Patient 1

Patient 2

Normal age-related range (clinical laboratory)

Lymphocytes

Absolute CD3+

2394

2830

850–3200 cells/µL

Absolute CD3+CD4+

1406

2065

400–2100 cells/µL

Absolute CD3+CD8+

1178

766

300–1300 cells/µL

CD4+/CD8+ ratio

1.1 (L)

2.5

1.5–2.5

B cells (CD19+)

122

266

120–740 cells/µL

% class-switched memory B cells

2% (L)

4% (L)

9–26%

NK cells

1102 (H)

133

7–480 cells/µL

CD3+CD4–CD8– alpha/beta

0.6%

0.8%

<1.5%

    

Normal range *

CD4+ T cells

CD4+ memory

(CD45RO+)

33.1

19.1 (L)

23.4–66.5

CD4+ TEM

21.5

11.4

9.1–48.4

CD4+ TCM

9.0 (H)

5.3

2.1–7.5

CD4+ TEMRA

2.1 (L)

1.8 (L)

3.0–13.6

CD4+ Teff

0.3

0.5

0–2.5

CD4+ naïve (CD45RA+)

59.1

74.2 (H)

21.2–62.7

CD8+ T cells

CD8+ memory (CD45RO+)

77.3 (H)

39.4

8.2–68.3

CD8+ TEM

30.0

14.7

0–30.7

CD8+ TCM

2.6 (H)

0.7

0.1–2.5

CD8+ TEMRA

43.6 (H)

20.7

8.7–29.0

CD8+ Teff

2.4

2.5

0–29.4

CD8+ naïve (CD45RA+)

15.2

54.6

8.3–83.9

T follicular cells

CD4+ CXCR5+

1.8 (L)

2.0 (L)

2.2–9.4

CD8+ CXCR5+

0.3

0.2

0–4.4

Treg cells

Total Treg (% of CD4+)

3.6

2.8

2.6–6.3

Naive Treg (CD45RO–)

27.1

38.9

15.1–49.4

Memory Treg (CD45RO+)

57.2

41.8

40–82

T cell cytokines

CD4+ TEM IFN-γ+

37.5

9.3 (L)

12.8–56.4

CD4+ TEM IL-13+

3.0

6.8

2.3–7.0

CD4+ TEM IL-17+

1.8

2.4

1.0–3.2

CD8+ TEM IFN-γ+

55.2

35.5 (L)

42.0–100

CD8+ TEM IL-13+

0.7

0.4

0.2–8.1

CD8+ TEM IL-17+

1.7 (H)

1.4 (H)

0–0.9

Checkpoint molecules

CD4+ TEM CTLA-4+

22.4 (L)

21.2 (L)

24.5–43.7**

CD4+ TEM PD-1+

58.5

44.9

26.6–70.3

CD4+ TEM TIM-3+

3.7 (H)

4.8 (H)

0.4–1.8

Activation marker

CD4+ TEM HLA-DR+

20.2 (H)

10.2 (H)

1.5–9.4

CD8+ TEM HLA-DR+

6.3

7.5

0–22.5

  1. Information on the optimized antibody panels used for flow cytometry can be obtained by contacting the corresponding author. Numbers represent the percentage of cells comprising each subset or percentage of cells expressing the indicated markers.
  2. TCM, central memory T cells; Teff, effector T cells; TEM, effector memory T cells; TEMRA, T cell re-expressing CD45RA; H, higher than normal range; L, lower than normal range
  3. *The normal range is represented by mean ± 2 × standard deviation of n = 7 healthy controls, mean age ± SD 34.3 ± 4.9 years and ** mean age ± SD 54.8 ± 10.8 years. Treg cells were defined as CD3+CD4+CD127low/–CD25highFoxP3+.