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Table 1 Clinical and laboratory characteristics of MDS patients withand without autoimmune disorders (AD)

From: VEXAS syndrome in myelodysplastic syndrome with autoimmune disorder

Characteristics

MDS with AD(n = 85)

MDS without AD(n = 429)

Total(n = 514)

P value

Sex(%)

   

0.024

 Male

37(43.5)

245(57.1)

282(54.9)

 

 Female

48(56.5)

184(42.9)

232(45.1)

 

Age,median(range),y

55(22–78)

57(7–84)

56(7–84)

0.538

WHO classification 2016(%)

   

0.719

 MDS-SLD

3(3.5)

19(4.4)

22(4.3)

 

 MDS-SLD-RS

2(2.4)

16(3.7)

18(3.5)

 

 MDS-MLD

46(54.1)

224(52.2)

270(52.5)

 

 MDS-MLD-RS

4(4.7)

11(2.6)

15(2.9)

 

 MDS-EB1

17(20.0)

79(18.4)

96(18.7)

 

 MDS-EB2

9(10.6)

63(14.7)

72(14.0)

 

 MDS with isolated del(5q)

0

6(1.4)

6(1.2)

 

 MDS-U

4(4.7)

11(2.6)

15(2.9)

 

Hb,median(range),g/L

79(41–155)

80(26–165)

80(26–165)

0.535

WBC,median(range), × 109/L

2.8(0.32–27.95)

2.78(0.41–25.45)

2.79(0.32–27.95)

0.786

ANC,median(range), × 109/L

1.14(0–6.52)

1.16(0–20.53)

1.16(0–20.53)

0.658

PLT,median(range), × 109/L

60(6–487)

62(2–603)

61(2–603)

0.912

BM blast,median(range),%

2.25(0–18)

2.5(0–19.5)

2.5(0–19.5)

0.618

CD4/CD8 T cell ratio

1.35(0.42–5.91)

1.67(0.37–8.42)

1.59(0.37–8.42)

0.032

Serum IL-6,pg/ml

6.22(2–31.5)

5.19(2–188)

5.28(2–188)

0.904

Serum TNF-α,pg/ml

21.6(4–1000)

14.9(4–478)

16.3(4–1000)

0.104

IPSS-R karyotype(%)(n = 439)

   

0.341

 Very good

0

3(0.8)

3(0.7)

 

 Good

36(50)

216(58.9)

252(57.4)

 

 Intermediate

25(34.7)

86(23.4)

111(25.3)

 

 Poor

4(5.6)

19(5.2)

23(5.2)

 

 Very poor

7(9.7)

43(11.7)

50(11.4)

 

Trisomy 8 positive(%)

22(30.6)

45(12.3)

67(15.3)

0.001

IPSS-R risk group(%)(n = 439)

   

0.558

 Very low

0

12(3.3)

12(2.7)

 

 Low

21(29.2)

109(29.7)

130(29.6)

 

 Intermediate

27(37.5)

114(31.1)

141(32.1)

 

 High

15(20.8)

77(21.0)

92(21.0)

 

 Very high

9(12.5)

55(15.0)

64(14.6)

 
  1. MDS-SLD, MDS with single lineage dysplasia; MDS-RS-SLD, MDS with ring sideroblasts with single lineage dysplasia; MDS-MLD, MDS with multilineage dysplasia; MDS-RS-MLD, MDS with ring sideroblasts with multilineage dysplasia; MDS-EB1, MDS with excess blasts-1; MDS-EB2, MDS with excess blasts-2; MDS-U, MDS unclassifiable; Hb, haemoglobin; WBC, white blood count; ANC, absolute neutrophil count; PLT, platelet count; BM, bone marrow; IL-6, interleukin-6; TNF-α, α-tumor necrosis factor; IPSS-R, Revised International Prognostic Scoring System