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. 2020 Jun;105(6):e264-e267.
doi: 10.3324/haematol.2019.223305. Epub 2019 Oct 3.

Clonal hematopoiesis in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis

Christopher Maximilian Arends  1 , Marlene Weiss  2   3 , Friederike Christen  1 , Claudia Eulenberg-Gustavus  2 , Anthony Rousselle  2 , Ralph Kettritz  2   3 , Kai-Uwe Eckardt  3 , Willy Chan  1 , Kaja Hoyer  1 , Mareike Frick  1 , Lars Bullinger  1   4 , Markus Bieringer  5 , Adrian Schreiber  6   3 , Frederik Damm  7   4
Affiliations

Clonal hematopoiesis in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis

Christopher Maximilian Arends et al. Haematologica. 2020 Jun.
No abstract available

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Figures

Figure 1.
Figure 1.
Sequencing analysis. (A) Spectrum of somatic gene mutations found in our cohort of 112 patients with ANCA-associated autoimmune vasculitis (AAV). Genes marked with an asterisk are only covered by the custom panel (Online Supplementary Methods). (B) Prevalence of clonal hematopoiesis of indeterminate potential (CHIP) according to age groups. Patients with a single mutation are represented in light blue, patients with multiple mutations in dark blue. (C) Comparison of CHIP prevalence in the AAV cohort with prevalences in previously described cohorts. Error bars depict the 95% confidence intervals. (D) Longitudinal quantification of variant allele frequencies (VAF) in selected patients. Only patients with a relevant increase or decrease of VAF over time are shown. The therapy regimens administered are depicted as colored bars on the x-axis. Relapse is represented by triangles. UPN: unique patient number; AZA: azathioprine; CYC: cyclophosphamide; MTX: methotrexate; MMF: mycophenolate mofetil; RTX: rituximab.
Figure 2.
Figure 2.
Functional data. Individual data points are depicted in scatterplots and summarized in boxplots. (A) Neutrophil oxidative burst detection using the DHR assay; depicted is the stimulation index SI = mean channel fluorescence of stimulated versus unstimulated cells, (B, C) Neutrophil membrane expression of CD177 or PR3 measured on isolated neutrophils by flow cytometry using anti-NB1 or anti-PR3 antibodies, depicted as expression index EI (B) or percentage of mPR3- and CD177-positive cells (C). EI = (MFIstimulated cells- MFIunstimulated cells) /MFIunstimulated cells. (D) mRNA expression measured in PB leukocytes with qPCR. (m)PR3: (membrane-)proteinase 3; MPO: myeloperoxidase; RUNX3: Runt-related transcription factor 3; JMJD3: jumonji domain-containing protein 3; DHR: dihydrorhodamine; NB1: neutrophil-specific antigen; PB: peripheral blood; EI: expression index; SI: stimulation index; MFI: mean fluorescence intensity.
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References

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