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. 2023 Jul 26;7(8):e934.
doi: 10.1097/HS9.0000000000000934. eCollection 2023 Aug.

Recovery of Bone Marrow Function in VEXAS Syndrome-potential Role for Romiplostim

Adam Al-Hakim  1 , Alyssa Cull  2 , Joanna Topping  3 , Fatima Nadat  1 , Joanna Milek  2 , Razan Alhefzi  2 , Michael F McDermott  3 , Roger Owen  4 , Catherine Cargo  4 , James Poulter  5 , David G Kent  2 , Sinisa Savic  1   3
Affiliations

Recovery of Bone Marrow Function in VEXAS Syndrome-potential Role for Romiplostim

Adam Al-Hakim et al. Hemasphere. .
No abstract available

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Conflict of interest statement

DK is a Scientific Editor for HemaSphere. The remaining authors have no conflicts of interest to disclose.

Figures

Figure 1.
Figure 1.
Summary of clinical progress and treatment responses. Timeline of symptoms and complications development, investigations, and treatment responses. BM = bone marrow; Bx = biopsy; CRP = C-reactive protein; CT = computer tomography; DVT = deep vein thrombosis; LCV = leukocytoclastic vasculitis; PLT = platelets; PMR = polymyalgia rheumatica; PUO = pyrexia of unknown origin; RBC = red blood cells.
Figure 2.
Figure 2.
Biological response to romiplostim. (A) Viably frozen PBMCs from 4 patients were thawed and stained with the following antibodies: CD34 PE (clone 561, Biolegend), CD38 FITC (clone HIT2, BD Biosciences), CD90 APC (clone 5E10, BD Biosciences), FLK2 BV650 (clone 4G8, BD Biosciences), CD10 APC-Cy7 (clone HI10a, Biolegend), CD45RA V450 (clone HI100, BD Biosciences), and 7AAD (Thermo-Fisher). Samples were analyzed on a MoFlo Astrios (Beckman Coulter). (C) In-house flow cytometry assay, for quantification of ASC/NLRP3 protein specks in sera. Briefly, 100 μL of patients’ sera was incubated in Phycoerythrin conjugated anti-ASC (TMS-1) Antibody (653904, BioLegend) and APC-conjugated NLRP3 Antibody (IC7578A, BioTechne) for 1 hour at room temperature, shaking. Totally 50 μL of sample was measured by using a Cytoflex S Flow Cytometer, gating for events 1 μM in size, using Flow Cytometry Submicron Particle Size Reference beads (F13839, Thermo-Fisher Scientific). Results were analyzed using CytExpert software and presented as ASC positive events per microliter. Data are represented in a box and whisker plot of ASC/NLRP3 protein specks. PBMC = peripheral blood mononuclear cells.
See this image and copyright information in PMC

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