Figure 3.
Kaplan Meier estimates of the combined field and clinic cohorts. (A) OS of combined cohorts: OS of complete cohort (field and clinic cohort, n = 231, black line) and divided by sex: 87 females (green line) and 144 males (blue line), P < .01. (B) OS according to inheritance pattern. Red line shows autosomal dominant/nonTINF2 (AD, n = 112); turquoise line shows autosomal recessive/X-linked recessive (AR/XLR, n = 63). Purple line indicates disease due to TINF2 (n = 25), and gray shows 31 patients with unknown genotype (AD vs AR/XLR P < .01, AD vs TINF2 P < .01, AR/XLR vs TINF2 P = .33). (C) OS of AD-nonTINF2 vs all other inheritance pattern groups: nonTINF2 AD disease (dark red, n = 112) vs all other inheritance pattern groups (AR/XLR/TINF2, blue, n = 88 P < .01). (D) OS compared with transplant-free survival. OS of 231 patients with telomere biology disorders (black line), with 77 of 231 receiving either HCT, liver transplant, and/or lung transplant. Transplant-free survival is indicated by the green line. One patient receiving a kidney transplant was not included in the calculations. Observed survival (stair-step lines) are shown; shaded areas show 95% confidence intervals. AD, autosomal dominant; AR, autosomal recessive; XLR, X-linked recessive.

Kaplan Meier estimates of the combined field and clinic cohorts. (A) OS of combined cohorts: OS of complete cohort (field and clinic cohort, n = 231, black line) and divided by sex: 87 females (green line) and 144 males (blue line), P < .01. (B) OS according to inheritance pattern. Red line shows autosomal dominant/nonTINF2 (AD, n = 112); turquoise line shows autosomal recessive/X-linked recessive (AR/XLR, n = 63). Purple line indicates disease due to TINF2 (n = 25), and gray shows 31 patients with unknown genotype (AD vs AR/XLR P < .01, AD vs TINF2 P < .01, AR/XLR vs TINF2 P = .33). (C) OS of AD-nonTINF2 vs all other inheritance pattern groups: nonTINF2 AD disease (dark red, n = 112) vs all other inheritance pattern groups (AR/XLR/TINF2, blue, n = 88 P < .01). (D) OS compared with transplant-free survival. OS of 231 patients with telomere biology disorders (black line), with 77 of 231 receiving either HCT, liver transplant, and/or lung transplant. Transplant-free survival is indicated by the green line. One patient receiving a kidney transplant was not included in the calculations. Observed survival (stair-step lines) are shown; shaded areas show 95% confidence intervals. AD, autosomal dominant; AR, autosomal recessive; XLR, X-linked recessive.

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