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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