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Clonal hematopoiesis is associated with progression of idiopathic pulmonary fibrosis
Lori Asarian, Jianlong Jia, Dmytro Sirokha, Lara Paulini, Daniela Dietel, Mircea Gabriel Stoleriu, Marion Frankenberger, Ali Önder Yildirim, Katharina S. Götze, Juergen Behr, Gary M. Hunninghake, Isis E. Fernandez
Lori Asarian, Jianlong Jia, Dmytro Sirokha, Lara Paulini, Daniela Dietel, Mircea Gabriel Stoleriu, Marion Frankenberger, Ali Önder Yildirim, Katharina S. Götze, Juergen Behr, Gary M. Hunninghake, Isis E. Fernandez
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Research Letter Aging Pulmonology

Clonal hematopoiesis is associated with progression of idiopathic pulmonary fibrosis

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Abstract

Authors

Lori Asarian, Jianlong Jia, Dmytro Sirokha, Lara Paulini, Daniela Dietel, Mircea Gabriel Stoleriu, Marion Frankenberger, Ali Önder Yildirim, Katharina S. Götze, Juergen Behr, Gary M. Hunninghake, Isis E. Fernandez

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Figure 1

CH mutations are associated with IPF progression and loss of lung function.

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CH mutations are associated with IPF progression and loss of lung functi...
(A) Rapid IPF progression is more prevalent in patients with CH mutations (n = 89 patients, 51 without CH and 38 with CH; Fisher’s test, **P < 0.01, median with min/max data). (B) The 12-month FVC loss was significantly greater in patients with CH mutations (n = 41 without CH and n = 28 with CH, Fisher’s test; **P < 0.01, median with min/max data). (C) Age did not significantly affect the presence of CH mutations. (D) Overall, DNMT3A was the more prevalent mutation (binomial test, ***P < 0.001). (E) TET2 mutations predominated in rapidly progressing patients (Fisher’s exact, **P < 0.01, unadjusted). (F) Frequencies and mutation types with representative median percentage VAF. (G) Protein positions of coding mutations by gene. Orange bars indicate functional domains, and mutations are shown as individual or multiple stacked events at the same position: magenta indicates high-impact mutations, blue indicates CH mutations, and green indicates rapidly progressive patients.

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