Fig. 1: The HSPC compartment in patients with VEXAS syndrome is less enriched in phenotypically primitive cells and skewed toward myelopoiesis.
From: Mechanisms of hematopoietic clonal dominance in VEXAS syndrome
a–d, Number of total hematopoietic cells (a), myeloid cells (b), lymphoid cells (c) and HSPCs (d) per microliter in BM aspirates of patients with VEXAS syndrome and age-matched controls assessed by multiparametric flow cytometry (n = 19 and 7). The median is shown with the interquartile range (IQR). Data on age-matched reference controls from ref. 59 are shown. e, Ratio between the number of myeloid and lymphoid committed progenitors from patients (n = 19 and 7). The median is shown with the IQR. Data on age-matched reference controls from ref. 59 are shown. f, Number of total hematopoietic circulating cells per microliter in the blood of patients with VEXAS syndrome and age-matched controls (n = 19 and 7). The median is shown with the IQR. Data on age-matched reference controls in e–g from ref. 59 are shown. g, Number of circulating HSPCs from different subpopulations per µl in the blood (n = 19 and 7). The median is shown with the IQR. h, Percentage of UBA1mut cells in the BM (left, n = 6, 5, 4 and 4) and peripheral blood (PB; right, n = 7, 6, 7 and 6) of different patients across hematopoietic subpopulations. Symbols and colors identify different patients. The median is shown with the IQR. i, Percentage of human cells in the BM of hematochimeric mice 12 weeks after transplantation of CD34+ HSPCs from healthy donors (n = 3) or patients with VEXAS syndrome (n = 3). Symbols and colors identify different donors of HSPCs. VEXAS HSPCs were transplanted without dilution with healthy donor HSPCs. j, Number of cells obtained on in vitro differentiation of HSPCs from patients with VEXAS syndrome (n = 2) and age-matched controls (n = 3). k, Number of erythroid and myeloid colonies obtained on seeding of 1,000 HSPCs from VEXAS PT1 (n = 1). The percentage represent the proportion of mutant colonies. For all panels, the Mann–Whitney U-test was used. *P < 0.5; **P < 0.01; ***P < 0.001; ****P < 0.0001.
