Bone marrow aspirate smear of a VEXAS syndrome patient
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http://datadryad.org/dataset/doi%253A10.5061%252Fdryad.6m905qfzx
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This dataset contains the digital slides of the bone marrow aspirate smears of a VEXAS syndrome patient from year 0, year 5, and year 7.
VEXAS syndrome is an autoinflammatory syndrome caused by somatic mutations in UBA1, a gene coding for the ubiquitin-activating enzyme. The disease was first defined in 2020 by Beck et al[1]. The patients develop refractory systemic inflammation, which can manifest as relapsing polychondritis among others. They also develop haematological symptoms, most frequently macrocytic anaemia, and the presence of vacuoles in the affected cells in the bone marrow is a key feature.
In the article[2] linked to this dataset, we provided the table (Supplementary Table S1) of the change in the numbers and the percentages of the vacuole-positive cells by maturation stage and by lineage in the bone marrow from three bone marrow aspirate examination over seven years. We scanned the smear slides to share with the scientific community.
[1]Beck DB, Ferrada MA, Sikora KA, Ombrello JC, Pei W, Balanda N, et al. Somatic mutations in UBA1 and severe adult-onset auto-inflammatory disease. N Engl J Med 2020;383:2628-38.
[2]Sakuma M, Tanimura A, Yasui S, Ishiguro K, Kobayashi T, Miyazaki H, et al. Case of polychondritis-onset refractory organising pneumonia with cytopaenia diagnosed as VEXAS syndrome: the disease course of seven years. Rheumatology 2021. doi: 10.1093/rheumatology/keab349
Methods
Bone marrow aspirate smears were obtained by Akira Tanimura and the differential cell counts with vacuole-positive cells were produced by Yusuke Ohshiro. The procedures, excluding the vacuole counts, followed the Japanese standard procedure. The counterpart standard procedure in the English literature is provided in the following article:
Lee SH, Erber WN, Porwit A, Tomonaga M, Peterson LC; International Council for Standardization In Hematology. ICSH guidelines for the standardization of bone marrow specimens and reports. Int J Lab Hematol. 2008 Oct;30(5):349-64. doi: 10.1111/j.1751-553X.2008.01100.x. PMID: 18822060.
https://onlinelibrary.wiley.com/doi/full/10.1111/j.1751-553X.2008.01100.x
Since uploading the original aspirate smear slides are impossible, we scanned the slides with NANO ZOOMER S360 (Hamamatsu Photonics). Scanning was performed by Maki Sakuma and Go Ogura.
创建时间:
2021-04-22



